(AIRP 2010) Vol. 1 Chest

Chest Radiology
10
An Approach to Diffuse Lung Disease, Sarcoidosis
Jeffrey R. Galvin, MD
Describing Diffuse Lung Disease

x The Alveolar vs. Interstitial Problem
x Alveolar or Interstitial?
Radiologic-Pathology Continuum
[Figure 1]
Figure 3
Chronic diseases tend

to involve the upper
lung zones secondary
to interaction with
lung lymphatics.
Figure 1
Radiology and pathology form a continuum of
visualization.
An Approach to Diffuse Lung Disease

x Radiograph
Plain Film and CT Opacities
¾Lung volumes
x Nodules
¾Opacity
x Reticulation and Lines
¾Distribution
x Ground glass
¾

x Consolidation
x Computed tomography
x Cystic airspaces
¾Opacity
¾Distribution Plain Film and CT Opacities
x Nodules
Lung Volumes ¾Sarcoid
x Reduced [Figure 2- left]
¾Silicosis, coal-workers
¾Pathology distal to the airway
¾Hypersensitivity Pneumonitis
¾Fibrosis
¾Metastasis
¾IPF, asbestosis, sarcoidosis, chronic
x Reticulation and Lines
hypersensitivity pneumonitis
¾Fibrosis
x Increased [Figure 2 - right]
IPF-lower, subpleural
¾Pathology of the airway
Asbestosis-lower, subpleural
¾Emphysema, asthma, bronchitis,
Sarcoidosis-peribronchovascular
constrictive bronchiolitis, LAM
Chronic hypersensitivity
pneumonitis - mid and upper lung
zone
x Ground glass [Shah and Miller AJR 2003]
¾
Airspace, interstitial, combined
DIP, NSIP, AIP, DAD (32%)
Infection (32%)
Drug toxicity (11%)
Hemorrhage (3%)
Ground glass with reticulation
Fibrosis
x Consolidation
Figure 2 ¾Organizing Pneumonia (BOOP)
Left: Fibrosis results in reduced lung volumes. ¾Chronic eosinophiic pneumonia
Right: Airways disease results in increased lung ¾Lymphoma
volumes. ¾Bronchoalveolar cell carcinoma
¾Infection
Distribution: Upper vs Lower ¾Hemorrhage
[Figure 3]
x Cystic airspaces
An Approach to Diffuse Lung Disease 12 Chest Radiology

¾Mimics reticulation on plain Edema
radiographs x Centrilobular [Figure 6 right]
¾Fibrosis and honeycombing Airway related
IPF - Lower, subpleural x Panlobular [Figure 7 left]
LAM - Diffuse ¾
LCH - Upper x Septal
¾Lymphatic
Radiologic - Pathology Continuum Ca, lymphoma, sarcoidosis
[Figures 7 right and 8 left]
Anatomy - Secondary Lobule Edema
x

x Random [Figure 8 right]
¾Polygonal ¾Hematogenous spread of tumor
¾1-2.5 cm ¾TB
¾Smallest unit demarcated by
connective tissue septa
x Most useful diagnostically
¾

HRCT
Gross examination
Histologic section
¾Explains HRCT appearance
Broad range of lung diseases
Especially interstitial disease
Anatomy - Secondary Lobule

[Figures 4 and 5]
x Core structures
¾Axial interstitium
¾Bronchiole
¾Pulmonary artery Figure 5
¾Lymphatics Septal pattern on HRCT and gross specimen.
x Septal structures
¾Peripheral interstitium
¾Pulmonary veins
¾Lymphatics
x Parenchyma
¾Alveolar interstitium
¾Alveoli
¾Pulmonary capillary bed
Figure 6
Figure 4
Left: Bronchovascular Pattern.
The secondary lobule Right: Centrilobular Pattern.
with lymphatics in
the interloblular
septa and along the
bronchovascular
bundle.
Abnormal Patterns
x Bronchovascular
¾Bronchus
Asthma, CF, bronchitis,
bronchiectasis [Figure 6 left]
¾Lymphatic Figure 7
Left: Panlobular Pattern.
Ca, lymphoma, sarcoidosis Right: Septal Pattern.
Chest Radiology 13 An Approach to Diffuse Lung Disease

Sarcoidosis: Laboratory Abnormalities
x BAL
¾Ç Macrophages, È proportions; Ç
CD4 helper cells
x Angiotensin-Converting Enzyme
¾

macrophage
¾Ç 33-90%
x Hypercalcemia 10%
x Hypercalciuria 30%
¾!
"
sources of 1-25 Dihydroxyvitamin

x Anergy
Figure 8
x Hypergammaglobulinemia
Left: Combined septal and bronchovascular pattern. Sarcoidosis: Respiratory System
Right: Random nodule pattern.
[Figure 9]
Sarcoidosis x 100% lung involvement
x Multisystem granulomatous disorder x Portal of entry
x Unknown etiology ¾Local lymph nodes
x Young and middle aged adults ¾Distant organs
x Bilateral hilar lymphadenopathy, x Disease distribution

¾Alveolar wall
lesions ¾Secondary lobule,
x

¾Axial CT
by evidence of noncaseating epithelioid ¾Radiograph
granulomas
x Exclusion of granulomas of unknown
cause and local sarcoid reactions
ATS Statement on Sarcoidosis 1999.
Sarcoidosis: Epidemiology
x Worldwide
¾Both sexes, all races, all ages
x Predilection for adults
¾Under 40 years
¾Peak 20-29 years
x U.S. prevalence
¾10 per 100,000 exams
x Highest disease
¾African-American women
Sarcoidosis: Clinical Features

x Asymptomatic
¾15-50%
x Constitutional symptoms
¾33%
x Dyspnea, cough, chest pain
¾33-50%
x Palpable lymph nodes
x 33-75%
x Ocular involvement
¾11-83%
x Cutaneous involvement
¾20-30% Erythema nodosum, Lupus
pernio
Figure 9
Sarcoidosis Pathogenesis.
Non-Caseating Granuloma and Fibrosis
Alveolar Distribution

Sarcoidosis and the Secondary Lobule Parenchymal Disease: Radiography
[Figure 10] x Bilateral
x Symmetrical
x Nodules
x Reticulonodular
x Masses
x Ground Glass
x Hilar Retraction
x Bulla
x Honeycombing
Sarcoidosis: Adenopathy
[Figures 12 and 13]
x Node Group CXR CT
x Hilar 84 88
x R. Paratrach 76 100
x A-P Window 72 92
x Subcarinal 12 64
Figure 10 x Ant. Med. 12 48
x Post. Med. 0 16
Bronchovascular distribution of granulomas in
sarcoidosis.
Sarcoidosis: Computed Tomography

[Figure 11] Figure 12
x Nodules
Lymph node
x Masses
involvement is
x Ground Glass a hallmark of
x Fibrosis sarciodosis.
¾Conglomeration
¾Distortion
x Emphysema
x Bulla
x Honeycombing
Figure 13

lymph nodes are
common.
Sarcoidosis: Staging Based on

Adenopathy and Parenchyma
Left: Distribution of nodules in sarcoidosis.
Right: Masses in sarcoidosis.
Sarcoidosis Stage I
Figure 11 Left: Ground glass in sarcoidosis.

Sarcoidosis Stage II
sarcoidosis.

Sarcoidosis Stage III Sarcoidosis: Differential Diagnosis
x Infection
Sarcoidosis Stage IV ¾Tuberculosis, Fungal (Histoplasmosis)
x Pneumoconiosis
Sarcoidosis Progression ¾Silica, Beryllium
x Hypersensitivity Pneumonitis
Sarcoidosis and the Parenchma - x Malignancy
Computed Tomography ¾Lymphoma
x Thickened bronchovascular bundles
x Nodules Miliary Tuberculosis
¾Peribronchovascular
¾Pleural, subpleural and septal Transbronchial Spread of Tuberculosis
x Consolidation and large nodules
x Ground-glass opacities Histoplasmosis
x Fibrosis
Silicosis
Thickened Bronchovascular Bundles
[Figure 14]
Berylliosis
Extrinsic Allergic Alveolitis
Sarcoidosis: Mortality
x Mortality range 5-10%
x

x Cardiac arrhythmia
x Pulmonary hemorrhage
¾Aspergilloma
Cor Pulmonale
Sarcoidosis: Cardiac Involvement

x Clinical involvement 5%
¾Heart block, arrhythmia, mitral
regurgitation, CHF (dilated
cardiomyopathy) and sudden death
Figure 14
x Autopsy involvement 20-30%
Peribronchovascular opacities in sarcoidosis.
x Localized wall motion abnormalities
Peribronchovascular Nodules ¾Anterior and apical
¾MRI, Echocardiograph, Thallium-201
Peribronchovascular and Pleural Vignaux AJR 184 Jan 2005.
Nodules
Cardiac Sarcoidosis [Figure 15]
Septal Lines
Ground Glass Opacities
Consolidation and Large Nodules
Fibrosis
Bronchovascular Bundle Distortion

[Figure 10]
Conglomerate Mass
Fibrosis and Emphysema
Fibrosis and Honeycombing
Sarcoidosis: Diagnosis
x Typical clinical and radiologic Figure 15
manifestations

x Non-caseating granulomas zones of increased signal on T2 and early gadolinium
images.
x Transbronchial Bx
x Endobronchial Bx

Dilated Cardiomyopathy Sarcoidosis: Therapy
x Cardiac, CNS, eye involvement
Sarcoidosis: Mycetoma x Hypercalcemia
x Present in 40-50% of cystic lesions x Corticosteroids
¾Bullae, cavities or bronchiectasis ¾Relief of symptoms; resolution of
x Hemorrhage radiologic abnormalities; improved
x Steroids may convert to invasive process function
x Cytotoxic agents
Mycetoma [Figure 16] ¾Methotrexate, azathioprine
x Chlorambucil, cyclophosphamide,
antimalarials
x Risk of recurrence
Sarcoidosis Resolution
Sarcoidosis: Prognosis
x Favorable
¾Acute onset, erythema nodosum,
¾> 80% spontaneous remission
¾Löfgren syndrome
¾Low stage
x Poor
¾Chronic course, Lupus pernio
¾Older age at presentation
¾# !
Figure 16
¾Black race, extrathoracic involvement
Mycetoma in a cystic space caused by sarcoidosis.
Sarcoidosis Conclusion
Reference
General
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4. Epler GR, McLoud TC, Gaensler EA, Mikus JR Carrington CB. Normal chest roentgenograms in chronic
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6. Felson B. A new look at pattern recognition of diffuse pulmonary disease. American Journal of

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7. Calvin JR. Mon M, Stanford W. High-resolution computed tomography and diffuse lung disease. Curr Probl
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12. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, lchikado K, Ando M, Nakamura H.
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14. Mayo JR. Webb WR, Gould R, Stein MG, Bass I, Gamsu G, Goldberg H. High- resolution CT of

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19. Murata K, Itoh H, Todo G, Kanaoka M, Noma 5, Itoh T, Furuta M, Asamoto H, Torizuka K. Centrilobular

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20. Murata K, Khan A, Rojas KA, Herman PG. Optimization of computed tomography technique to

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21.

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22. Muller NI, Miller RR. Computed tomography of chronic diffuse lung disease. American Review of

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23. $

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Sarcoidosis
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28. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu C, Maclntyre NR, Coleman RE, Putman CE. $
correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology
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30. Gleeson FV, Traill ZC, Hansell CM. Evidence of expiratory CT scans of small- airway obstruction in
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32. Kuhlman JE, Fishman EK, Hamper UM, Knowles M, Siegelman SS. The computed tomographic spectrum of
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37. Newman ES, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul
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41. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol
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42. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis
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The Idiopathic Interstitial Pneumonias
The Idiopathic Interstitial Pneumonias The Idiopathic Interstitial Pneumonias
Chronic Diffuse Lung Disease x Liebow 1975
[Figures 1 and 2] x Supporting lung structures
x Alveolar involvement ¾'
¾Surrounding airways ¾Fibrosis
¾

x

Alveolar wall x Initiated within the airspace
Alveolar space Liebow, Prog Resp Dis 1975.
x Restrictive physiology
x Decreased lung volumes The Idiopathic Interstitial Pneumonias
x Increased attenuation Current List-ATS/ERS Concensus
x Subacute or chronic C
¾Weeks to months x Idiopathic Pulmonary Fibrosis (IPF)

¾Usual Interstitial Pneumonia (UIP)
x Respiratory Bronchiolitis-Interstitial Lung
Disease (RB-ILD)
x Desquamative Interstitial Pneumonia
(DIP)
x Acute Interstitial Pneumonia (AIP)
x Cryptogenic Organizing Pneumonia (COP)
x $
'

Q$'V
Figure 1
Travis, et al. Am J Respir Crit Care 2002.
The idiopathic
interstitial Idiopathic Pulmonary Fibrosis
pneumonias x
'

!
involve the pattern

alveolar walls
and spaces. x 5th-7th decade
¾66% > 60 years
¾^<KKKKK
*

<K<KKKKK
men
x Insidious onset of dyspnea
¾6 months before diagnosis
¾Restrictive ventilatory defect
¾Rales and clubbing
x
¾Cigarette smoke
¾~
:
+
*
dust, metal dust

¾}|
"
¾Autoantibodies common (ANA, RA)
Figure 2
x Median survival 2.5-3.5 years
The lung Usual Interstitial Pneumonia: Histology

volumes are x Geographic variation
low and there x Temporal variation
are areas of
increased ¾Fibroblast foci
density. ¾

x |":

x '
¾Minimal
¾No correlation outcome
x Abnormal wound healing
x Prognosis
¾Fibroblast foci
Presence and extent
Katzenstein, Am J Respir Crit Care Med 1998.

22 Chest Radiology
Idiopathic Pulmonary Fibrosis: Imaging 48%
[Figure 3] Relevance to NSIP
x Radiograph abnormal-95% x Uncertain diagnosis
¾Volume loss x Discordant data
¾Reticulonodular opacities x Disease other than IPF
¾Lower lobe ¾Hypersensitivity pneumonitis
¾Honeycombing ¾Collagen-vascular disease
x Computed tomography ¾Infection
¾Peripheral and lower lobe Hunninghake, Am J Respir Crit Care Med, 2001.
¾Reticulation and ground glass
Progress to honeycombing IPF Rad-Path Discord
¾Ground glass in areas of traction
bronchiectasis Smoking Related ILD Interstitial Lung
Hartman, Chest 1996. Disease [Figure 5]
x Respiratory bronchiolitis
¾RB
x Respiratory bronchiolits-interstitial lung
disease
Figure 3 ¾RB-ILD
x Desquamative interstitial pneumonia
The abnomalities ¾DIP
are predominantly
peripheral and
lower lung
"
progressive
volume loss.
IPF-Progressive Volume Loss

Figure 5
Idiopathic Pulmonary Fibrosis
[Figure 4] Smoker’s
Macrophages.
Figure 4
Typical
peripheral
recticulation
and
honeycombing
and traction
bronchiectasis
in a patient
!"
Smoking Related ILD - RB

x Clinical
IPF and Emphysema
¾Cigarette smoke or equivalent
Utilility of Biopsy for Diagnosis of IPF ¾Asymptomatic
x Prospective, multi-center study x Pathology
¾91 patients suspected of IPF ¾Peribronchiolar macrophages
x Clinical diagnosis ¾!

¾Positive predictive value with a x Imaging

Z^ ¾Centrilobular nodules
x Imaging diagnosis

JX
¾Positive predictive value with a Upper lobe predominance

= x Ground glass opacity
¾CT always abnormal in patients with x Bronchial wall thickening
proven IPF x Decreased attenuation
x Histologic diagnosis x Emphysema
¾Agreement regarding the presence or x Air trapping
absence of IPF-85% x Reticulation
¾Agreement in patients without IPF- Remy-Jardin, Radiology 1993.
Chest Radiology 23

Smoking Related ILD - RB-ILD ¾Cysts
[Figure 6] Alveolar ducts
x Clinical Bronchioles
¾Cigarette smoke or equivalent Emphysematous spaces
¾Dyspnea Park, J Comput Assist Tomogr 2002.
¾Restrictive or mixed PFT’s
¾Good Prognosis Desquamative Interstitial Pneumonia
x Pathology [Figure 7]
¾Peribronchiolar macrophages
¾!

x Imaging
¾Centrilobular nodules
Figure 7

JX
Upper lobe predominance Ground glass
x Ground glass opacity opacities in
x Bronchial wall thickening #"
x Decreased attenuation
x Emphysema
x Air trapping
x Reticulation
Remy-Jardin, Radiology 1993.
Dependent Density
RB and DIP
Smoking Related ILD [Figure 8]
Figure 6
Small
centrilobular
nodules with
an upper lobe
predominance
$%#"
Figure 8
Smoking related intestitial lung disease with upper

&

Smoking Related ILD - DIP emphysematous spaces combined with lower lobe
x Clinical ground glass.
¾Cigarette smoke
¾4th and 5th decade
¾Uncommon
¾70% survival - 10 years
Acute Interstitial Pneumonia - AIP
¾Steroids
x Hammon-rich disease
x Pathology
x Rapidly progressive
¾Pigmented macrophages
x Days-weeks
¾'

x

Plasma cells and eosinophils
x Mean age 50 years
¾Fibrosis
x 50% fatal at least
x Imaging
¾Ground glass Vourlekis, Medicine 2000.
Symmetrical
Basal predominance
¾Reticulation

24 Chest Radiology
Acute Interstitial Pneumonia: Histology Acute Interstitial Pneumonia: Computed
x Exudative phase Tomography
¾Hyaline membranes x Exudative phase
¾Edema ¾Consolidation
¾' ¾Bilateral
x Collapse of alveoli ¾Focal sparing
x Organizing phase x Organizing phase
¾Type II hyperplasia ¾Distortion
¾]
¾Traction bronchiectasis
x Diffuse alveolar damage ¾Ground glass
Katzenstein, Am J Pathol 1986; chikado, AJR Johkoh, Radiology 1999.
1997.
chikado, et al. Am J Respr Crit Care Med 2002.
Diffuse Alveolar Damage
Acute Interstitial Pneumonia [Figure 11]
Acute Interstitial Pneumonia:

Radiography [Figures 9 and 10]
x Diffuse
x

x Costal sparing
x Mechanical ventilation
x Resembles ARDS
Figure 11
Focal areas of
sparing are

*"
Figure 9
*+
+
5 lobes.
Cryptogenic Organizing Pneumonia

x

x Pattern of repair
x Self-perpetuating
x Cryptogenic
x Secondary
¾Connective tissues disease,
hematologic malignancy, drugs or
organ transplantation
x Focal
¾Bacteria, legionella, mycoplasma,
mycobacterial, or infarction
Lohr. A'(()"
Figure 10
Most patients x Terminology problem
are intubated
with diffuse ¾Bronchiolitis obliterans OP (BOOP),
opacities. bronchiolitis obliterans (BO),
bronchiolitis interstitial pneumonia
(BIP)
x Subacute presentation (3 months)
x M=F
x Cough, dyspnea, weight loss, fever
x Restrictive PFT’s
x Steroid responsive
x Relapse common
Epler. NEJM 1985.
Chest Radiology 25

Cryptogenic Organizing Pneumonia:
Histology
x Fibroblastic plugs in alveoli
x Fibrosis in the alveolar space
x May be airway centered
¾Bronchiolitis

Radiography [Figure 12]
x Consolidation
¾Unlateral or bilateral
x Small nodules
¾10-50%
x Lung volumes
¾Normal in 75%
Figure 13
/ <

consolidation. The differential includes chronic
eosinophilic pneumonia, bronchoalveolar cell
Figure 12 carcinoma, lymphoma and infection.
Cryptogenic
organizing
pneumonia is
characterized
by focal areas
of consolidation
more common
in the lower lung
"
Figure 14
Diffuse nodules
Computed Tomography may also be
x Consolidation 90% seen in COP.
x Ground glass 75%
x Bronchial thickening and dilatation
x Small nodules along bronchovascular
bundles
x Large nodules (15%)
¾Irregular margins
¾Air bronchograms
x Reverse Halo

[Figures 13 and 14] N
Interstitial Pneumonia:
Histology
N
Interstitial Pneumonia x 3 categories
x Katzenstein ¾Cellular
¾Described in 1994 ¾Fibrosing
x ~

+
!
'' ¾Mixed
¾UIP, RB-ILD, DIP, OP, AIP x

x Represents a variety of etiologies x OP common
¾Collagen vascular disease, drug x Temporally uniform
reaction, inhaled antigen
¾Inadequately sampled UIP or OP
x Median age 45
x Onset gradual with wide range
¾6 months to 3 years
x Better prognosis
Katzenstein, Am J Resp Crit Care 1994.
Nicholson, Am J Respir Crit Care Med, 2001.

26 Chest Radiology
N
Interstitial Pneumonia:
Imaging
x Few reports on chest radiography
x Wide variety of CT patterns
¾Ground glass, consolidation, reticular
and honeycombing
x /
!

x CT pattern indistinguishable
¾UIP 32%
¾Hypersensitivity 20%
¾OP 14%
¾Other 12%
Hartman, Radiology, 2000.
N
Interstitial Pneumonia
NSIP ATS Consensus Conference Figure 15

x Pathologists <$=

+>"
x Radioiologists
x Pulmonalogist Idiopathic Pulmonary Fibrosis
x 300 cases submitted [Figure 16]
¾11 cases agreed to be NSIP by all
pathologists
x Imaging
¾Lower lobe
¾Peribronchiolar reticulation and
distortion
¾Subpleural clearing
N
(NSIP) Current View
NSIP Fibrosis with IPF Imaging

x Areas of NSIP commonly found in proven
cases of UIP
x NSIP and UIP Figure 16
¾Different severity of injury?
¾Different mechanism of injury? !"
x Prognosis in these cases is driven by the
imaging RB/RB-ILD [Figure 17]
Katzenstein AA, et al. Amer J of Surg Path 2002.
Hypersensitivity Pneumonitis
NSIP in Cigarette Smokers
N
OP-NSIP [Figure 15]
The Idiopathic Interstitial Pneumonias:

Current List
x Idiopathic Pulmonary Fibrosis (IPF)
¾Usual Interstitial Pneumonia (UIP)
x Respiratory Bronchiolitis-Interstitial Lung Figure 17
Disease (RB-ILD) $%#"
x Desquamative Interstitial Pneumonia
(DIP)
x Acute Interstitial Pneumonia (AIP)
x Cryptogenic Organizing Pneumonia (COP)
x $
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Chest Radiology 27

RB-ILD/DIP [Figure 18] Organizing Pneumonia [Figure 21]
Figure 18 Figure 21
#" Organizing Pneumonia.
Acute Interstitial Pneumonia NSIP in the Literature

[Figures 19 and 20]
NSIP-IPF
NSIP-Cigarette Smokers [Figure 22]
Figure 19
*/"
Figure 22

@
/+

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="
NSIP-Hypersensitivity Pneumonitis
NSIP-Organizing Pneumonia [Figure 23]
Figure 20
*
?

"
Figure 23
Patients with organizing pneumonia may have a

/
="

28 Chest Radiology
NSIP-Organizing Pneumonia [Figure 24]
Figure 24
Patients with organizing pneumonia may have a biopsy

="
References
General
1.
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Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors,
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304.
2.

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IPF/UIP
3. #
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\ Thorac Imaging
<==@<<Q_VJX<_=&
4. q
]

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'!

:
+
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<==Z@<`^Q_

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5. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic

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&
!
<==X@<KXQV<ZKZ
12.
6. ]*
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! &

<=^`@Z<XX&
7. Tobin RW, Pope CE, 2nd, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of
!
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*!
!

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<==Z@<`ZQV<ZK_Z&
8. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D.
'

:

! !
/
:
! &
Radiology 1990;176(755-9).
9. Coxson HO, Hogg JC, Mayo JR, Behzad H, Whittall KP, Schwait DA, Hartley PC, Galvin JR, Wilson JS,
# !
$&

+
!

!

! &

\

<==^@<``Q`V<_=`&
10. Gay SE, Kazerooni EA, Toews GB, Lynch UP, 3rd, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork MA,
!
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:!

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'!

!

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<V<KX^J&
11. [
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|*

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$!
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<==Z@<`^Q<V<==
203.
DIP
12. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl U Med 1966; 274(3)113-
28.
13. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia

!

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DAD/Alp
14. [
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Chest Radiology 29

15. ~
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16. }
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17. Ichikado K, Johkoh T, Ikezoe U, Takeuchi N, Kohno N, Arisawa U, Nakamura H, Nagareda T, Itoh H,

&

! !
/

*!
! &

<==^@<ZQJVXXXZ&
18. Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda 0, Tomiyama

#&

!
/

X
&

<===@J<<QXVZ`=X&
19. Katzenstein AL, Myers UL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and

&

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<=Z@<KQ_VJ`^&
20.

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#!

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21.
$]
#
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<==X@<ZZQXVZ<^JK&
NSIP
22.
j
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+

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&

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24.
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\#
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! !
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BOOP/Organizing Pneumonia
25. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple

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<==Z@<^KQJVJ=<`&
26. Carlson BA, Swensen SJ, O’Connell EJ, Edell ES. High-resolution computed tomography for obliterative
!&

<==X@ZQXVXK^Z&
27. Chandler PW, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of
bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol
<=Z@<_^Q`VZ===K&
28. Epler GR, Colby TV, McLoud TC, Carrington CB, Oaensler EA. Bronchiolitis obliterans organizing
&

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<=Z`@X<JQXV<`JZ&
29. Gosink RB, Friedman Pd, Liebow AA. Bronchiolitis obliterans. Roentgenologic¬pathologic correlation. Am J

/!

<=^X@<<^Q_VZ<XJ&
30. Haddock JA, Hansell DM. The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol
<==J@`Q^^V^_ZK&
31. Katzenstein AL, Myers JL, Prophet WD, Corley LS, 3rd, Shin MS. Bronchiolitis obliterans and usual

&

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32. ]
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8.
33. ]
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34. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD.
Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern

<==^@<`^Q<JV<XJX=&
35. McLoud TC, Epler GR, Colby TV, Gaensler EA, Carrington CB. Bronchiolitis obliterans. Radiology
1986;159(1)1-8.
36. Muller NL, Cuerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC. Differential

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30 Chest Radiology
Airways Disease: The Movement from Anatomic to Physiologic
Assessment
Assessment of Dyspnea - A Common
Clinical Problem
x 55 million adult smokers
x 15 million meet criteria for bronchitis
x 5 million with airway obstruction
x 10 million with asthma
Gordon Snyder Figure 1
Differential Diagnosis of Airways Small airway

tethered to the
Obstruction pleural surface by
x Common alveolar walls.
¾Emphysema, bronchitis,
bronchiectasis, asthma
x Uncommon
¾LAM, BO, panbronchiolitis, sarcoid,
!<

[
Diseases with Obstructive Physiology
The Changing Role of Imaging

x Diagnosis
x Functional assessment
Why Pulmonary Functions are

Insensitive
x PFT’s based on wide range of normal
¾80-120% predicted
x Diseases with opposing physiologic
processes Figure 2
Mosaic attenuation on an expiratory CT in patient with
The “Silent Zone” of the Lungs constrictive bronchiolitis.
[Figures 1 and 2]
“Small Airways”
x Peter Macklem
¾1970’s
x No cartilage
¾< 2mm physiologists
Figure 3
¾1mm pathologists
x Tethered Airways involvement
¾Fiber skeleton at the level of the
¾Pleura secondary lobule.
Weibel.
Diffuse Lung Disease - Airways [Figure 3]

x Airways involvement
x Obstructive physiology
x Increased lung volumes
Airways Disease 34 Chest Radiology

Airways Disease - Direct Signs [Figure 4] ¾!<

x Changes ¾Lower lobes
¾Airway wall x Distal Acinar
¾Airway lumen ¾Paraseptal
x Opacities ¾Distal acinus
¾Tubular ¾Subpleura
¾Nodular ¾Pneumothorax
¾Branching
Figure 5
Cigarette
Figure 4 smoke related
“Tree-in-bud” in emphysema is
a patient with a most severe in
respiratory infection. the upper lobes.
Airways Disease - Indirect Signs

x Mosaic density
¾Air trapping Emphysema - Clinical Presentation
x Subsegmental atelectasis x Cough, dyspnea and sputum production
x Ground glass x Hemoptysis rare
¾

Airways Disease x $

*

x Emphysema ¾After age 50, 20-30 years of smoking
x Emphysema and Fibrosis x Cor Pulmonale (late) related to hypoxemia
x !<
and loss of capillary bed
x Langherhans Cell Histiocytosis
x Bronchiectasis Emphysema and Cor Pulmonale
x Asthma
x Allergic Bronchopulmonary Aspergillosis Emphysema - Pulmonary Functions
x Sarcoidosis x Important to identify patients at risk
x Diffuse Panbronchiolitis x Reduction in Fev1
x Bronchiolitis obliterans ¾Most reproducible
x Lymphangioleiomyomatosis x RV increases followed by TLC
x j

*
Emphysema - ATS D

¾Insensitive to early changes
x Permanent enlargement of airspaces x Diffusing capacity
distal to the terminal bronchiole, ¾$:

accompanied by destruction of the walls x Small airways tests
*!
:

Emphysema - Radiographic Feature
Emphysema x #
¾Concave diaphragm
Emphysema - C
[Figure 5] ¾Increased A-P diameter
x Proximal Acinar ¾Retrosternal airspace
¾Centrilobular x

¾Resp bronchiole x Bulla
¾Cigarette smoke ¾Cystic airspaces > 1cm
¾Upper lobes x Radiography is insensitive
x Panacinar ¾41% of moderate disease
¾Entire acinus ¾66% of severe disease
Chest Radiology 35 Airways Disease

Saber Trachea [Figure 6]
Figure 8
Repiratory
Bronchiolitis.
Figure 6
Saber Trachea.
Emphysema and Computed Tomography

[Figure 7]
Relationship of RB and Emphysema
x Prospective study
x 111 subjects
¾Followed for 5 years
Imaged at inception T0 and 5
years T1
¾Smokers, nonsmokers and quitters
x Micronodules at T0 predisposes to the
development of emphysema at T1
x Micronodules and emphysema at T0
predicts more rapid decline in lung
function
Remy-Jardin Radiology 2001.
Relationship of RB and Emphysema

Remy-Jardin, Radiology 2001.
Figure 7
Typical low attenuation lesions of emphysema. Emphysema and Fibrosis
x 14 patients
The Diagnosis of Mild Emphysema - x Scanning electron microscopy
Correlation of CT and Pathology Scores x Thick and thin walls
x HRCT detects emphysema ¾[!

¾[+
!

*

Nagai & Thurlbeck. Am Rev Respir Dis 1985.

PFT’s
x HRCT excludes emphysema Emphysema and Fibrosis
¾Patients with moderate to severe x Normal lung volumes and
*
x
*

Kuwano et. al. Am Rev Respir Dis 1990. x Reduced diffusing capacity
¾Severe
Early Emphsema x Minimal pulmonary reserve
Respiratory Bronchiolitis - “Smoker’s Emphysema and Fibrosis

Bronchiolitis” [Figure 8] x TLC 119%
x Common change x VC 126%
¾All smokers x RV 109%
x Pigmented macrophages x |j<j

ZZ
¾In respiratory bronchioles x ~j

JZ
¾Surrounding alveoli
x Upper lobe predominance
x Usually asymptomatic
¾May cause symptoms

Emphysema and Fibrosis [Figure 9]
Figure 11
Figure 9 Typical Nodules in LCH.

Emphysema and Fibrosis.
Idiopathic Pulmonary Fibrosis
Langerhans Cell Histiocytosis - Clinical

Presentation
x Almost exclusively cigarette smokers
x Slight male preponderance
x Cough and dyspnea most common
x May be asymptomatic
x Occasional bone lesion
Langerhans Cell Histiocytosis -

Histology [Figure 10]
x Nodular
¾Interstitial lesions
¾Located near bronchioles Figure 12
x Histiocytes, eosinophils, Cytic Lesions LCH.
x plasma cells and lymphocytes
x Diagnosis requires Langerhans Cell Histiocytosis -
x Langerhans cells Radiographic Features [Figure 13]
¾Large histiocytes x Varies over time
¾Folded nuclei x Upper lobe
¾Eosinophilic cytoplasm ¾Predominance
x Path DDX x Nodules
¾Eos pneumonia, DIP, UIP ¾0.5-1.0 cm in upper lobes
¾Early
x Cysts replace nodules
¾Later
x Honeycomb lung
x Pneumothorax 15%
Figure 10 x Adenopathy and effusion are unusual
The range of

Langerhans Cell
Histiocytosis.
Figure 13
LCH is characterized by
low attenuation areas
with bizarre shapes.
Langerhans Cell Histiocytosis

[Figures 11 and 12]

Langerhans Cell Histiocytosis - EM and Bronchiectasis - Pathophysiology
Immunohistochemistry x Dilatation of bronchi
x Immunoperoxidase staining x Reversible form
¾CD1a, S-100 protein ¾Infection
x Cells in clusters in interstitium ¾Atelectasis
x EM x Congenital
¾X-bodies ¾Tracheobronchomalacia
¾Langerhans cell granules x
¾Birbeck granules x Post-obstructive
x Fibrotic
Langerhans Cell Histiocytosis - Clinical ¾IPF
Course ¾Sarcoid
x Clinical resolution
¾Common Williams-Campbell [Figure 15]
x Radiographic abnormalities
¾Persist
x Occasional progression
¾Fibrosis and honeycombing
x May be fatal
¾Rapid progression
Langerhans Cell Histiocytosis
Alpha-1 Antitrypsin D

Pathophysiology
x 1-2% of emphysema in the US
x Alpha-1 antitrypsin inactivates neutrophil
elastase
x Production controlled by 2 genes
x Level of antitrypsin dependent on allele
x ZZ homozygotes most severe Figure 15
x Smoking accelerates the destruction
Williams-Campbell.

Imaging Features
x Radiograph may be normal
x Lower lobe predominance Mounier-Kuhn Syndrome
x Panacinar emphysema
x CT Bronchiectasis - P

¾Upper lobe involvement x Primary Ciliary Dyskinesia
¾Bronchiectasis ¾Kartagener’s
¾Airway thickening common x '
x CT more sensitive x Post-infectious
¾TB, Measles, pertussis, viral
x Post-toxic bronchitis
[Figure 14] ¾Gastric acid aspiration
x Immunologic
¾ABPA
Primary Ciliary Dyskinesia
Post Obstructive Bronchiectasis

x Neoplasm
x Foreign body
x Broncholith
x Lymph node enlargement
Figure 14
*$'//"

Post Obstructive Bronchiectasis RB/RB-ILD [Figure 18]
[Figure 16]
Figure 18
$%#"
Emphysema and Fibrosis [Figure 19]

Figure 16
Post obstructive bronchiectasis in a patient with
mucoepidermoid carcinoma.
Bronchiectasis - Clinical Presentation

x Cough
x Purulent sputum
x Hemoptysis (50%)
x Dyspnea
x Rare
¾Clubbing, brain abscess, amyloidosis
Bronchiectasis - Radiographic Features

x Prominent markings
x Crowding of Vessels Figure 19
x “Tram Tracks” Emphysema and Fibrosis.
x Loss of volume
x Cystic spaces Langerhans Cell Histiocytosis
[Figures 20 to 22]
Bronchiectasis - CT Features
x Bronchi in the periphery
x “Signet Rings”
x “Tram Tracks”
x Sensitivity
¾Collimation
Emphysema [Figure 17]
Figure 20
Early LCH Nodules.
Figure 17
>

@ / "

x 6% in the American population
¾Rate has doubled in 20 years
¾Higher incidence in large cities
Asthma - Extrinsic
x Family history atopy
x Early onset <30 years
x Seasonal symptoms
x Increased IGE
x Positive skin tests
x Often remits
Figure 21
Asthma - Intrinsic
Late LCH Cysts and Nodules x No atopy
¾Absence of external triggers
x Older age group
x Increased blood eosinophils
x Increased sputum eosinophils
x Fixed airway obstruction
¾Progressive
Asthma - Pathology
x Airway smooth muscle
¾Hypetrophy
x Airway wall
¾'
¾Edema
x Airway plugging
Figure 22 ¾Mucus
End-stage LCH. ¾'
"

[Figure 23] Asthma - Radiographic Features
[Figure 24]
x Chest roentgenogram
¾Often normal
x Airway thickening
¾Chronic disease
x Rapid attenuation of vessels
¾Hypoxemia
x Pneumomediastinum
¾Pneumothorax
x #
¾Adaptive
¾Later air trapping
Silva AJR 183 September 2004.
Figure 23
Lower lobe predominance in Alpha-1 antitrypsin.
Bronchiectasis
Diffuse Lung Disease - Airways

Asthma - ATS D

x Reversible airway disease

x Increased airway responsiveness
x
*

chronic asthmatic Figure 24

¾Why? Airway thickening in asthma.

Asthma - H

[Figure 25] Allergic Bronchopulmonary Aspergillosis
- Presentation & Pathology [Figure 27]
x Atopic individuals
¾Most common
x

x *

Figure 25
¾Fungus
Severe ¾Inspissated mucous
/Q

in which you
x Presentation with
can see the ¾Cough, fever
slips of the ¾Hemoptysis
diaphragm as ¾Worsening asthma
it inverts. x Seen in stable asthmatics
x Good response to steroids
Asthma - CT Features [Figure 26]

x More sensitive than CXR
x Reversible
¾Consolidation
¾Atelectasis Figure 27
¾Mucoid impaction
¾Airway narrowing ABPA represents
¾Air trapping a non-invasive
colonization of the
x Permanent
airways.
¾Bronchial wall thickening
¾Bronchiectasis
¾Emphysema
Allergic Bronchopulmonary Aspergillosis

- Imaging [Figures 28 amd 29]
x Bifurcating opacities
¾}:
¾

*
x Central Bronchiectasis
x

x Pleural disease
¾Uncommon
Figure 26
Mucoid impaction in severe asthma.

- Primary Criteria
x Asthma
x Eosinophilia
x Immediate skin test reactivity
x Precipitating antibodies (IgG)
Figure 28
x Elevated serum (IgE)
x
Mucoid impaction in ABPA.
x Central bronchiectasis

x Computed tomography
¾Centrilobular nodules
¾Branching opacities
¾Mosaic attenuation
Diffuse Panbronchiolitis [Figures 30]
Figure 29
Central bronchiectasis in ABPA.
Sarcoidosis and the Airways -

Computed Tomography
x Functional evidence of airways obstruction
¾Obstructive PFT’s are common
x |!

x Obstructive physiology correlates with
¾Decreased attenuation on expiratory
scans (small airways) Figure 30
¾

:

Early Diffuse Panbronchiolitis represented by
disease (large airways) widespread airways nodules.
Hansell et al. Radiology 1998.
Endobronchial Granulomas Diffuse Panbronchiolitis - Imaging Late

[Figure 31]
Small Airway Distortion x Radiography
¾Nodules
Reticular Pattern and Fibrosis ¾Cysts and bulla
¾#
Diffuse Panbronchiolitis x Computed tomography
x Japan most common ¾Centrilobular nodules
¾

!
|

¾Bronchiolectasis
North America ¾Bronchiectasis
¾HLA BW54
¾
J<
x Presentation
¾Dyspnea
¾Cough
x Obstructive PFT’s
x Slowly progressive
¾15 yr mean survival
x Erythromycin
¾May not be an antibacterial effect Figure 31
Diffuse Panbronchiolitis - Pathology Severe airway

x Discrete nodules involvement in
panbronchiolitis.
x |

¾Interstitium
¾Respiratory bronchioles
¾Alveolar ducts
¾Foamy histiocyte, lymphocyte and
plasma cells
x Late secondary ectasia
¾Proximal terminal bronchioles
Diffuse Panbronchiolitis - Imaging Early

x Radiography
¾Nodules 5mm
¾#

Constrictive Bronchiolitis - Introduction Constrictive Bronchiolitis - Imaging
x Confusing terminology [Figures 33 and 34]
¾Obliterative Bronchiolitis x #
¾Bronchiolitis Obliterans ¾Localized
¾Bronchiolitis Obliterans Organizing ¾Diffuse
Pneumonia x Discrete nodules
Different disease ¾Airway associated
Cryptogenic organizing pneumonia x Mosaic pattern
x Small airways x Airway thickening
¾Fibrosis x Bronchiectasis
¾' x Air trapping
x Response to
¾'

¾Infectious disorders
Figure 33
Constrictive Bronchiolitis - Clinical
Presentation Mosaic
x Cough, dyspnea and malaise attenuation
x History in constrictive
bronchiolitis.
¾Prior infection
¾Exposure
x Hypoxemia
x Airway obstruction
Constrictive Bronchiolitis - C
x Infection
¾RSV, adenovirus and mycoplasma
x Toxic inhalation
¾Ammonia, acid and NO
x

Figure 34
x
j

x Organ transplantation Central
x Unknown bronchiectasis
and mosaic
Constrictive Bronchiolitis - Histology attenuation
[Figure 32] in constrictive
bronchiolitis.
x Obstruction
¾Terminal bronchiole
¾Respiratory bronchioles
x
+

x

¾Lymphs
¾Plasma cells
¾Histiocytes
Swyer-James Syndrome [Figure 35]
Figure 32
Constrictive
Bronchiolitis.
Figure 35
%>/

Swyer-James.
Right: Swyer-James Syndrome.
Swyer-James Syndrome - Adenovirus

Lymphangioleiomyomatosis - Clinical Lymphangioleiomyomatosis -
Presentation Radiographic Features
x Exclusively women x Reticulonodular opacities
x Reproductive years ¾Basilar
x Progressive dyspnea x Lung volume
x Chylous pleural effusions ¾Normal
x Hemoptysis ¾Increased
x Massive hemorrhage x Pleural effusion
¾60-75%
Lymphangioleiomyomatosis - Function x Pneumothorax
x Obstructive defect x Honeycombing late
x FEV1 is decreased
x TLC and RV increased Lymphangioleiomyomatosis - CT
x DLCO reduced Features [Figure 37]
x Hypoxemia x Thin-walled cysts
x Hypocapnia ¾
:
!

x Diffuse
Lymphangioleiomyomatosis - Histology x Bilateral involvement
x Smooth muscle proliferation x Adenopathy
¾Disorderly
¾Bronchioles, alveolar septa, arteries,
veins and lymphatics
x $

¾Air trapping
Lymphangioleiomyomatosis - Gross
Features [Figure 36]
x Cysts Figure 37
¾0.2-2cm
x Diffuse involvement Thin-walled
x Enlarged thoracic duct cysts and a
x Enlarged lymph nodes pneumothorax
in patient with
lymphangioleio-
myomatosis
Lymphangioleiomyomatosis [Figure 38]
Figure 36
Right: Typical thin-walled cyst in
lymphangioleiomyomatosis.
Left: The upper and lower lobes are equally involved in
LAM.
Figure 38
%* / /

/ "

Lymphangioleiomyomatosis - Therapy
and Prognosis
x Slowly progressive course
¾Variable
x Progression
¾Cor Pulmonale
¾
+
x 50-80% 5 year survival
¾Average survival 10 years
x Hormonal therapy
¾Oophorectomy, progesterone
Tuberous Sclerosis [Figure 39]
Figure 41
ABPA.
Sarcoidosis [Figure 42]
Figure 39
LAM may represent partial expression of tuberous

sclerosis.
Figure 42
Sarcoidosis airways involvement.
Emphysema
Emphysema and Fibrosis Diffuse Panbronchiolitis [Figure 43]
Langherhans Cell Histiocytosis

Asthma [Figure 40]
Figure 43
Diffuse Panbronchiolitis.
Figure 40
Asthma.
ABPA [Figure 41]

Constrictive Bronchiolitis [Figure 44] Physiologic Measurement - An Integral
Part of Imaging
x Imaging provides physiologic information
¾Not available from pulmonary
functions
x

*

Airways Disease
x Emphysema
x Emphysema and Fibrosis
x !<

x Histiocytosis-X
x Bronchiectasis
x Asthma
x Allergic Bronchopulmonary Aspergillosis
Figure 44 x Sarcoidosis
Mosaic attenuation in constrictive bronchiolitis. x Diffuse Panbronchiolitis
x Bronchiolitis Obliterans
x Lymphangioleiomyomatosis
Swyer-James Syndrome
Diffuse Lung Disease - Airways
LAM [Figure 45]
Figure 45
/%*
References
General
1. Hartman T, Primack 5, Lee K, Swensen S, Muller N. CT of bronchial and bronchiolar diseases.
}!
<==_@<_==<<KKX&
2. Hogg JO, Macklein PT, Thurlbeck WM. Site and nature of airway obstruction in chronic obstructive lung
&

|
\

<=Z@J^ZQJ`V<X``K&
3. King GO, Muller NE, Pare PD. Evaluation of airways in obstructive pulmonary disease using high-

! &

\

<===@
<`=QXV==J<KK_&
4. Lucidarme O, Coche E, Cluzel P, Mourey-Gerosa I, Howarth N, Grenier P. Expiratory CT scans for chronic
*

*!

+

&
\

\

<==Z@<^KQJV<K<^&
5.
/&

*
!

&

\

<=^J@`JQV^J<_&
6.
|

&
~
+
!
!
/

!!
&

<==`@<=Q<VX<J&
7. Naidich D, McCauley Dl, Khouri NF, al e. Computed tomography of bronchiectasis. Journal of Computer

/ !
<=ZJ@_X^__&
8. Neeld DA, Goodman LR, Gurney JW, Greenberger PA, Fink JN. Computerized tomography in the evaluation
+

!
&

:*
+

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of the lung surface area in emphysema using computed tomography. Am J Respir Crit Care Med
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19. Kinsella M, Muller NE, Abboud RT, Morrison NJ, DyBuncio A. Quantitation of emphysema by computed
tomography using a “density mask” program and correlation with pulmonary function tests. Chest
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20. Klein JS, Gamsu G, Webb WR, Golden JA, Muller NE. High-resolution CT diagnosis of emphysema in
symptomatic patients with normal chest radiographs and isolated low diffusing capacity. Radiology
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21. Kondoh Y, Taniguchi H, Yokoyama S, Taki F, Takagi K, Satake I Emphysematous change in chronic asthma

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22. Kuwano K, Matsuba K, Ikeda T, Murakami J, Araki A, Nishitani H, Ishida T, Yasumoto K, Shigematsu N.
The diagnosis of mild emphysema. Correlation of computed tomography and pathology scores. Am Rev

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23. Miller RR, Muller NE, Vedal S, Morrison NJ, Staples CA. Limitations of computed tomography in the

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41. Backman KS, Greenberger PA, Patterson R. Airways obstruction in patients with long-term asthma

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radiography and high resolution computed tomography of the lungs in asthma. Am Rev Respir Dis
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Inhalational Lung Disease
Pneumonokoniosis
x “It will then be necessary to embrace
under a single title all essentially identical
forms of disease
x …the pneumonokoniosis (from Konis,
dust) recommends itself” Figure 1
Zenker 1866 Hematite Mining. Particles less that
5 microns can be
Inorganic Dusts deposited beyond
x Silica the conducting
x Asbestos airways in the
alveolar spaces.
x Coal
x Iron
x Beryllium
Pneumoconiosis - The Accumulation

of Dust in the Lungs and the Tissue Airway Velocity - Inertial Impaction,
Reaction to its Presence Sedimentation and Diffusion
x Dust macules
x ~++

x Diffuse alveolar damage
x Alveolar proteinosis
x Giant cell (GIP)
x }

Types and Sizes of Common Aerosols
Particle Clearance - Cough,

Tracheobronchial and Alveolar
Transport [Figure 2]
x Most important
¾Deposition less critical
x Mucociliary escalator
¾Outer gel, inner liquid sol
x 90% of particles removed within 2 hrs
x Alveolar transport
Particle Deposition - Inertial ¾Dissolution, engulfed by macrophages,
Impaction, Sedimentation and Diffusion removed to lymphatics
[Figure 1]
x <KKKKJKKKK

x Deposition related to particle size
x >10 microns deposit in nasopharynx and
Figure 2
large airways (100%)
x 1-5 micron particles deposit in lung Macrophages
parenchyma (20%) remove
small
particles
to regional
lymph
nodes.

%# 52 Chest Radiology
Early Basal Deposition
Figure 6
Removal to Lymph Nodes
The
Physiologic Gradients - A lymphatics
[Figure 3] are driven by
hydrostatic
pressure.
Therefore
/ Q

is best in the
dependent
Figure 3 lung.
Alveoli in the
bases are
smaller than
those in the
apex. Removal to Lymph Nodes [Figure 7]
Figure 7
Physiologic Gradients - A
This explains
[Figure 4] the tendency
for chronic
diseases to be
upper lobe.
Figure 4
The smaller
alveoli in the
bases enlarge
to a greated
degree than
those in Tuberculosis
the apex.
Therefore Silicosis - Mineralogy

Q

is directed x Silicon
towards the ¾Element
bases. x Silica (SiO2)
¾Mineral
x Silicone
¾Synthetic polymer
Physiologic Gradients - Blood Flow
[Figure 5] Silicosis: Epidemiology
x Occupational exposure predominates
¾3 million workers
x Mining, stonecutting, engraving and
Figure 5 foundry work
There is x Males more commonly affected
increased x Degree of exposure underestimated

Q
x Increased risk of neoplasia and
hydrostatic
pressure in
scleroderma
the dependent
vessels. Silicosis: Pathogenesis
x `

+*
threshold
x <KK

+<KK
Physiologic Gradients - Lymphatic Flow affected

[Figure 6] x > 5 micron particle removed in nares and
. upper airways
x 80% of particles removed in hours to days
x Retained particles consistently .5 - .7
microns
Chest Radiology 53

%#
Silicosis: Pathogenesis
x Macrophages and polys concentrate
x Macrophages generate oxygen-free
radicals
x !

x Immune related tissue damage
¾Rheumatoid factor, ANA and gamma
globulin
Silicosis: Clinical Manifestations

[Figures 8 and 9]
x Diagnosis
¾Typical imaging pattern of adenopathy
and nodules
¾Exposure to high concentration of Figure 9
silica Left: Silicoproteinosis is an acute lower lobe process.
¾10-20 years of exposure Right: Silicosis predisoposes a patient to having active
x Simple silicosis tuberculosis.
¾Asymptomatic
x Symptoms with PMF
x Intense exposure
¾Silicoproteinosis
x TB and cancer
Figure 10
The silicotic
nodule
is typical
response to
inhaled silica.
Figure 8
%*
/

hallmark of silicosis.
Right: Nodules with an upper lobe predominance is
typical.
Simple Silicosis: Pathology [Figure 10]

x

X
q
¾

¾Mid-zone concentric collagen
¾Peripheral dust laden cells
Simple Silicosis: Imaging Manifestations

[Figure 11] Figure 11
x Adenopathy common Eggshell
x
<KJK
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x
`<K
¾Eggshell pattern
Simple Silicosis: Imaging Manifestations

x Well-circumscribed nodules
¾1-10 mm
x Upper lobe and posterior
¾Lymphatic gradient
¾CT more sensitive
x Pleural lesions
¾Candle-wax or pseudoplaques

Progressive Massive Fibrosis: Pathology Silicosis and Tuberculosis [Figure 14]
x Conglomeration of nodular lesions
x !

¾2 cm
x

¾1 cm
x Upper lung zones
¾Posterior
Progressive Massive Fibrosis: Imaging

Manifestations [Figure 12]
x Progression after exposure
x

¾Posterior
x Usually bilateral
x Carcinoma mimic
¾Solitary
¾Lower lobe
Figure 14
x Associated emphysema
¾Not always smoking related Tuberculosis in a patient with silicosis.
¾Scar emphysema
Silicosis: Computed Tomographic

Technique
x Thick sections of value in nodular diseases
Figure 12
¾Small nodules easier to differentiate
Progressive from vessels
massive x Thin sections 1-2 mm collimation at 10

" mm intervals or 3-5 selected images with
prior thick section CT
x High spatial frequency algorithm
x Supine
x No contrast
Silicotic Alveolar Proteinosis: Pathology Silica and Lung Disease

[Figure 13]
x Adenopathy
x High concentration of particulate silica x Nodules
x Acute onset x PMF
¾Weeks-months x Silicoproteinosis
x :

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x Tuberculosis
x Similar to surfactant x Cancer
x Type II cell hyperplasia
Asbestos: Introduction
x }
+

¾#

x Flexible and strong
x Corrosion, thermal and electrical
resistance
x 500 tons - 3 million tons
¾60 years
Figure 13
x 9 million people exposed
Crazy paving ¾Primary (mining)
pattern associated ¾Secondary (industrial)
with alveolar ¾Nonoccupational (air)
proteinosis.
Nonoccupational Exposure
Chest Radiology 55

%#
Asbestos Bodies [Figure 15] x Mesothelioma
x Indicates exposure x Asbestosis
x /

x Lung cancer
x Iron and mucopolysaccharide coat
x Predominantly amphiboles
x ]

¾< 20 microns not coated
¾

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¢

x Fibers cannot be removed
x Lower posterior disease
Figure 16
Left: Pleural effusions are the most common early

complication of asbestos exposure.
Right: Rounded atelectasis is usually preceded by a
pleural effusion.
Figure 17
Asbestosis is lower
lobe subpleural
Figure 15 process.
Left: Asbestos bodies a commonly

found in urban dwellers.
Right: *

macrophages and therefore cannot
be removed to regional lymph nodes.
Pleural Plaques: Pathology [Figure 18]

x

Asbestos
¾(50-10%)
Serpentine: Chrysotile x Dense bands of collagen
x 95% of commercial use
¾“Basket weave”
x Curly and pliable
x Asbestos bodies absent
x Textile manufacture
x

:

x Fragments easily
x Dose response
x Chemically unstable
¾Between parenchymal asbestos bodies
¾Dissolves easily
and presence of plaques
x Less pathogenic
x Pathogenesis uncertain
Asbestos
Amphiboles: Amosite, Crocidolite,
Anthophilite, Tremolite and Actinolite
x 5% of commercial use
x $ !

x Do not fragment easily
x ]

Q¥JK
V
¾Not cleared Figure 18
x More likely coated
x Higher carcinogenic potential Pleural Plaques.
Asbestos Related Chest Disease

[Figures 16 and 17]
x Pleural effusions
x Pleural plaques
x Round atelectsis
x Pleural thickening
¾Diffuse

Pleural Plaques Pleural Plaques [Figure 21]
x Postero-lateral parietal pleura
x Central diaphragm
x Absent
¾Apices and costophrenic angles
x Almost always bilateral
x Sharply demarcated
x Millimeters to 10 cm
x May calcify extensively
x Highly suggestive of asbestos exposure
Roberts, AJCP 1971.
Pleural Anatomy [Figure 19]
Figure 21
Visceral pleural plaques.
Diffuse Pleural Thickening

x Smooth pleural density
¾¢
¥
J`
+
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+
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wall
¾/
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craniocaudal
x Posteromedial lower lobes
x Involves costophrenic angle
x Mediastinal pleural involvement
¾Rare
Figure 19 ¾Suggests mesothelioma
x Visceral and parietal pleura
The visceral pleural stripe is best seen between the
ribs.
¾Adhesions
x Sequela of prior effusion?
Pleural Effusion: D

Pleural Plaques: Imaging x History of exposure to asbestos

x Radiography insensitive x
+
++
¾(8-40% of autopsy cases) ¾Imaging or thoracentesis
x Companion shadows x Absence of other disease related to
¾Fat and muscle effusion
x HRCT x Absence of malignant tumor for 3 years
¾[
:

Epler, JAMA, 1982.
Pleural Fat [Figure 20]
Pleural Effusion: Clinical Presentation
x Most common abnormality
¾First 20 yrs
x 3% prevalence
¾Asbestos exposed
x Small < 500 ml
x Serosanguinous
x Persist for weeks to 6 mos
x 66% asymptomatic
x 28% recur
Pleural Effusion: Differential Diagnosis

x Lung cancer
x Tuberculosis
x Benign asbestos effusion
x Mesothelioma
Figure 20
! /
"
Chest Radiology 57

%#
Round Atelectasis
x Described 1928 Loeschke
x

:

x Associated conditions
¾Asbestos exposure, CHF, infarct, TB
and histoplasmosis
x Preceded by effusion
Round Atelectasis: Histology

Figure 23
[Figure 22]
x '

!
+
!
:
Round
pleura Atelectasis.
x Extensive pleural folding beneath the

x Layers of invaginated pleura bound by

!
x $

Menzies, AJSP 1987.
Asbestosis: ATS Criteria 1986

x Reliable history of exposure
x Latency of at least 10 years
x Restrictive pulmonary functions
Figure 22 ¾DLco and VC <80%
Round x
!

atelectasis ¾Inspiratory crackles, clubbing
is associated x Chest radiographic abnormalities
with pleural
effusion.
¾']
+
¥
<K
Q

V
Asbestosis: Histology
x Early
¾Fibrosis of respiratory bronchioles
x Progression
¾Terminal bronchioles, alveolar ducts
and alveolar septa
x Minimum 2 asbestos bodies in area of

Round Atelectasis: Imaging Criteria Craighead, Arch Pathol Lab Med 1982.
[Figure 23]
Asbestosis: Chest Radiography
x Well-circumscribed x Lower lobe
x Round or oval opacity ¾Irregular opacities
x “Comet tail” sign ¾
x Pleural thickening ¾Associated pleural disease
x Volume loss x Large inter-observer variation
¾Low perfusion
Asbestosis: Pathologic D

x Normal in 26% of path proven cases

x '

¾Associated with asbestos bodies
x Biopsy
¾Not the standard of practice
Asbestosis
x Dose-response relationship
x Probable exposure threshold
x Latency period inversely proportional to
exposure level
x Latency is several decades
x Cigarette smoke may act synergistically

Asbestosis and Cigarette Smoking - Asbestosis vs UIP
Small Irregular Opacities x Asbestos exposure in the last 30 years is
x Small opacities are related to low
¾Dust exposure, cigarette smoke, age, x Clinical asbestosis requires substantial
radiographic technique and obesity exposure
x Cigarette smoke causes x Asbestos exposed individuals can have
¾'
other interstitial lung diseases
¾Emphysema x Band like opacities merging with the
¾Bronchiolar thickening pleura are rare in UIP
x Asbestos causes x
q

¾'
rare in asbestosis
x Asbestos workers who smoke Gaensler, ARRD 1991.
¾Have more opacities
Al-Jarad, Thorax 1992.
¾Related to dust exposure and
cigarettes Asbestosis: High-Resolution CT
x Short lines and parenchymal bands are
Asbestosis - High-Resolution CT

x Lower lobe and posterior
x Strong association with diffuse pleural
¾Reticulonodular opacities
disease
¾Parenchymal bands
x Multifocal
¾Curvilinear subpleural line
x #/

"
¾Interstitial short lines

individuals with normal radiographs and
¾Honeycombing
PFT’s
x High sensitivity
x Obstructive PFT’s correlate with
x
emphysema
x $

*!
¦
+
abnormalities Aberle, Radiology 1988.

x Prone imaging is key! Aberle, AJR 1988.
Parenchymal Bands [Figure 24]

Staples, ARRD 1989.
Asbestosis: Dependent Density

x

*
¾5X’s greater
x Posterior alveoli
¾Smaller or collapsed
¾Less steep ventilatory gradient
¾Closing volumes (10-40% of VC)
Asbestosis: Computed Tomographic

Technique
x 1.5-2 mm collimation
x 10 mm interval
x High spatial frequency algorithm
x Prone
x /!

Figure 24 Asbestos Related Chest Disease

Parenchymal bands and round atelectasis in a patient Tuberculosis
with asbestosis.
Silicosis
Reticulonodular Opacites Asbestosis
Curvilinear Subpleural Line Particle Deposition and Clearance:
Cough, Tracheobronchial and Alveolar
Short Lines Transport
Honeycombing
Chest Radiology 59

%#
Pulmonary Lymphoid Disorders
The Pulmonary Lymphoid System x BALT
x Lymphatics x Lymphoid aggregates
x Lymph nodes x Lymphocytes
x BALT x Dendritic cells
¾Bronchus associated lymphoid tissue x Langerhans cells
x Lymphoid aggregates
x Lymphocytes
x Dendritic cells
x Langerhans cells
The Pulmonary Lymphoid System

[Figure 1]
x Lymphatics
¾Originate in the pleura
¾Valves
¾Drain towards hilum
¾Follow interlobular septa
¾Accompany blood vessels
x Lymph nodes
x BALT
x Lymphocytes Figure 2
x Dendritic cells
x Langerhans cells Left: Lymphatic channels continue along the pulmonary
veins to the hilum. A second set of lymphatics
originates near the center of the secondary lobule and
follows the pulmonary arteries.
Right: These lymphatic channels continue along
the pulmonary veins to the hilum. A second set of
lymphatics originates near the center of the secondary
lobule and follows the pulmonary arteries.
Figure 1
Left: One set of lymphatics originate in the visceral

pleura as demonstrated by the lines on the surface of
the lung.
Right: These lymphatics enter the lung and follow the
interloblular septa in the periphery of the lung.
Pulmonary Lymphoid System [Figures 2 to 4]

x Lymphatics
x Lymph nodes
¾Encapsulated lymph nodes
Proximal bronchi Figure 3
Bifurcations Classic encapsulated intrapulmonary lymph nodes
¾Reactive lymph nodes

U$X

Peripheral and septal bronchi and are demonstrated adhering to the right
Cigarettes or dust main pulmonary.
Pulmonary Lymphoid Disorders 62 Chest Radiology

Pulmonary Lymphoid Disorders -
Derivations of BALT
x Hyperplasias of BALT
¾Follicular hyperplasia
Follicular bronchitis
Figure 4 ¾Diffuse hyperplasia
Lymphoid Interstitial Pneumonia
The majority of
intrapulmonary ¾Nodular Lymphoid Hyperplasia
lymph nodes Pseudolymphoma
are probably x Non-Hodgkin’s lymphomas
not visible ¾Low-grade B cell lymphomas
radiographically.
Almost all of
¾Lymphomatoid granulomatois
these lymph x Immune impairment
nodes are ¾PTLD, AIDS and other
subpleural and
inferior to the Koss. Sem Diag Pathol 1995.
carina.
Follicular Hyperplasias of BALT -
Hyperplasia of BALT [Figure 6]
x Follicular bronchitis and bronchiolitis
x Pathologic features
¾Antigenic stimulation of BALT
Reactive Lymph Nodes ¾Lymphoid aggregates
¾Peribronchial
The Pulmonary Lymphoid System ¾Reactive follicles
x Lymphatics
¾Minimal alveolar extension
x Lymph nodes
x Clinical
x BALT
¾Bronchus associated lymphoid tissue
x Lymphocytes
x Dendritic cells
Figure 6
x Langerhans cells
BALT - The Organizing Principle Follicular bronchitis

is characterized
[Figure 5]
by hyperplastic
x Lymphoid collections lymphoid follicles
¾Bronchial epithelium with reactive
¾Bifurcations germinal centers
x Absent in the normal adult distributed along
bronchioles and
¾Absent at birth to a lesser extent
¾Common in young children bronchi.
x Reappears with antigenic stimulation
¾Cigarette smoke
¾Connective tissue disease
¾AIDS
x Basis of pulmonary lymphoid disorders

Hyperplasia of BALT
Figure 5 x Clinical
¾Young adults (44 yrs.)
Bronchus associated ¾Cough and dyspnea
lymphoid tissue or Fever and weight loss
BALT is found along
the bronchiole, ¾'

interlobular septa and AIDS
"
/ Congenital
found only in young ¾Collagen vascular diseases
children. Sjogren’s
Rheumatoid arthritis
¾Uncertain etiology
Hypersensitivity reactions?
Chest Radiology 63 Pulmonary Lymphoid Disorders

Hyperplasia of BALT: Imaging [Figure 7]
x Radiography
¾Diffuse Figure 8
¾Reticulonodular
x CT %?/

¾Nodules 3-12 mm the alveolar septa.
Centrilobular
Peribronchial
¾Ground glass
¾Air trapping
Lymphocytic Interstitial Pneumonia -

Hyperplasia of BALT
x Pathologic Features
x Clinical
¾Women > men
¾4th-6th decade
¾Cough and dyspnea
¾Collagen vascular disease
Sjogrens, RA, and SLE
¾Bone marrow transplantation
¾AIDS rare in adults
Common in children
Figure 7 ¾Dysproteinemia
Almost all patients with follicular bronchitis have ¾Restrictive lung functions
centrilobular nodes that are less than 3mm. These Hyperplasia of BALT: Imaging
nodules correlate with the peribrochiolar distirbution of
hyperplastic lymphoid follicles shown on the histology [Figures 9 and 10]
section to your left. x Radiography
¾Lower lung zone
¾Reticulonodular
x CT
Follicular Hyperplasia - Differential ¾Ground Glass
Diagnosis ¾Nodules
x Respiratory bronchiolits Centrilobular
x Hypersensitivity pneumonitis

x Diffuse panbronchiolits ¾Cystic air spaces
x Cystic Fibrosis ¾Thickened BVB’s
x Primary ciliary dyskinesia ¾Adenopathy
Diffuse Hyperplasias of BALT -
x Lymphocytic Interstitial Pneumonia
¾:

Lymphocytes (T-cells)
Diffuse
¾Lymphoid follicles (B-cells)
Germinal centers
Peribronchial distribution
Spectrum with follicular
hyperplasia of BALT (Follicular
Bronchitis)
x Clinical
Figure 9
The lung windows demonstrate diffuse hazy ground
glass that correlates with diffuse alveolar wall
thickening. The alveolar wall thickening is primarily

/

"

Nodular Lymphoid Hyperplasia -
Hyperplasia of BALT
x Pseudolymphoma
x Clinical
¾Rare entity
¾Most cases were lymphomas
Monoclonal B cell proliferation
¾Middle age
Asymptomatic
¾Autoimmune Diseases 15%
Sjogren’s
SLE
Transverse myelitis
¾Surgical excision curative
Figure 10 Nodular Lymphoid Hyperplasia
Thin walled cysts are often found deep within the lung Hyperplasia of BALT: Imaging
parenchyma. Previous reports have suggested that [Figures 12 and 13]
airway narrowing or obliteration results in these cystic
lesions. x Radiography
The histology on the left demonstrates complete ¾Solitary Nodule

//

" ¾Focal Consolidation

/
// x CT
wall.
¾Air bronchograms
LIP vs Lymphoma 100%
¾Indistinct margins
LIP Lymphoma ¾Occasionally multiple
x Cysts 82% 2% ¾!

++

x Consolidation 18% 66% lymphoma

x Large Nodules 6% 41%
x Effusions 0 25%
Nodular Lymphoid Hyperplasia -

x Pseudolymphoma
¾Solitary, subpleural mass
¾Lymphoid proliferation
Interstitial
Perivascular
B and T cells
Polyclonal pattern
Benign
¾Reactive germinal centers
¾~+

+
!
x Clinical
Figure 12
The CT demonstrates the typical sub-pleural, solitary

lesion with indistinct margins.
The bulk of the lesion consists of a mass of lymphoid
tissue with multiple reactive germinal centers.
Figure 11
Nodular lymphoid
hyperplasia or
pseudolymphoma
presents as a solitary
subpleural mass of
lymphoid tissue with
numerous reactive
germinal centers.

Air bronchograms
Airway narrowing or “stretching”
Figure 13
Air bronchograms are universally present and the
/

/
the classical indistinct margin.
Figure 14
Pulmonary Lymphoid Disorders - Grossly low grade B-cell lymphoma usually presents
as a single white tan lesion that can be either well
Derivations of BALT circumscribed or indistinct.
x Hyperplasias of BALT This is well demonstrated by the gross specimen
x Non-Hodgkin’s lymphomas

*!+"&
¾Low-Grade B-cell lymphomas however, be multifocal as shown on the right and has
¾Lymphomatoid granulomatosis been reported as a primarily endobronchial lesion.
x Immune impairment
Primary Tracheal Lymphoma
Low-Grade B-Cell Lymphoma x Extremely rare
x Pathologic features x BALT derivative
¾] !
x Extensive at diagnosis
¾Small lymphocytes x Potentially curable
Alveolar wall
Peribronchiolar Lymphomatoid Granulomatosis
Perivascular [Figure 15]
¾Immunologic evidence of malignancy x Pathologic features
Monoclonality ¾Majority of cases are B-cell
B-cell marker CD20 lymphomas
¾Germinal centers ¾Reactive small T-cells
¾Malignant B-cells
Low-Grade B-Cell Lymphoma
+

x Clinical ¾Epstein-Barr virus
¾Similar presentation to nodular ¾

lymphoid hyperplasia ¾Necrosis
¾5th-6th decade Peribronchovascular
¾Male = Female Peripheral
¾Asymptomatic 50%
¾5 year survival 85-95%
¾Surgical resection
Rare recurrence
Low-Grade B-Cell Lymphoma [Figure 14] Figure 15

x Imaging
Lymphomatoid
¾Radiography Granulomatosis is an
$
angiocentric B-cell
Multiple lymphoma which
Consolidation often demonstrates
Air bronchogram areas of necrosis.
50%
Slow growth
¾CT
Consolidation

Lymphomatoid Granulomatosis Lymphoma and Immune Impairment
x Clinical [Figure 17]
¾7-85 years (mean 48 yrs) x Pathologic features
¾
QJ<V ¾B-cell non-Hodgkin’s lymphoma
¾Malaise and weight loss Driven by Epstein-Barr virus
¾Lung involvement 100% infection
¾Cough and dyspnea Diffuse polyclonal expansion
¾Skin 39-53% Reduced T-cell control
Nodules, ulcers and rash Malignant transformation
¾CNS 37-53%
¾Renal 32-40%
¾High mortality rate 53-90%
¾Most proceed to lymphoma
Figure 17
Lymphomatoid Granulomatosis
x Imaging Post transplant
¾Nodules 80% lymphomas
Multiple are driven by
Bilateral (80%) Epstein-Barr,
reduced T-cell
¾Mid and lower lobes surveillance
¾Cavitation 20% and malignant
¾Large masses transformation.
Correspond to infarcts Genetic
¾Diffuse reticulonodular opacities mutation may
eventually
¾Hilar adenopathy 25% result in
malignant
LYG [Figure 16] transformation
of one of
these clones,
represented in
purple (see web
pdf for color).
Lymphoma and Immune Impairment

x Clinical
¾Spectrum of benign to malignant
Infectious mono-like
PTLD polymorphic
PTLD monomorphic
¾Cyclosporin shortens induction
(<1 year)
¾May respond to reduction in
immunosuppression, anti-virals and
Figure 16 surgery
Chest CT on the left demonstrates a bronchovascular ¾Chemotherapy should be avoided
distribution of nodules that are shown to be areas of ¾Heart-lung up 20%
infarction on gross examination.
Lymphoma and Immune Impairment
x Imaging
¾Nodules
Pulmonary Lymphoid Disorders - May cavitate
Derivations of BALT Halo
x Hyperplasias of BALT Along bronchovascular bundles
x Non-Hodgkin’s lymphomas ¾Lymph node
x Immune impairment ¾Ground glass
¾Post-transplantation ¾Septa thickening
Lymphoproliferative disease (PTLD) ¾Consolidation
¾AIDS ¾Effusion
¾Other forms of prolonged immune
suppression

PTLD [Figure 18]
Figure 20
Figure 18
%/
/
`%{"
^_/
'//
who underwent a double lung transplant 7 years Nodular Lymphoid Hyperplasia
prior to presenting with shortness of breath, cough,
[Figure 21]
fever and chills for 2 months. The CT reveals
multiple multiple indistinct nodules in a characteristic
distribution along the bronchovascular bundles.
Bone Marrow Transplant - Prolonged

Chemotherapy
Pulmonary Lymphoid Disorders -

Derivations of BALT
x Hyperplasias of BALT
x Non-Hodgkin’s lymphomas
x Immune impairment
PTLD
AIDS
Other
Follicular Hyperplasia [Figure 19]

Figure 21
Nodular Lymphoid Hyperplasia which was formerly

known as Pseudolymphoma.
Low-Grade B-Cell Lymphoma [Figure 22]
Figure 19
Follicular Bronchitis.
Diffuse Hyperplasia of BALT - LIP

Figure 22
[Figure 20]
Low grade B-cell Lymphoma.

LYG [Figure 23]
Figure 23
Lymphomatoid Granulomatosis.
Immune Impairment - PTLD

[Figure 24]
Figure 24
Post Transplant Lymphoproliferative Disorder.
Immune Impairment - PTLD
BALT - The Organizing Principle

x Lymphoid collections
x Basis of pulmonary lymphoid disorders
References
1.
&

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@
<JQJV<`Z^<&
2. /:
~
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JKK<
~@`Q<JV=_^<&

Angiitis and Granulomatosis
x First characterized by Averill Liebow 1973
x Unknown etiology
x Angiitis
¾

+

:
x Granulomatosis
¾Necrosis of lung parenchyma not
related to blood vessel occlusion
Figure 1
Angiitis and Granulomatosis: Current
Vasculitis in the
List

x Wegener’s granulomatosis differential of
x Churg-Strauss syndrome vessel associated
¾Allergic granulomatosis nodules.
x Necrotizing sarcoid granulomatosis
x Bronchocentric granulomatosis
x Lymphomatoid granulomatosis
Angiitis and Granulomatosis: General

Concepts Wegener’s Granulomatosis: Classic
x Etiology remains unknown Pathology Triad
x '
:&
!+: x Vasculitis described 1852
x

~" ¾Von Rokitansky
x Adequate tissue samples are important x Wegener described 1936
x

+

¾Wegener’s
fungal x Focal vasculitis of
¾Arteries and veins
Pathogenesis of Vasculitis
x Necrotizing granulomas
¾Upper and lower airways
x Necrotizing glomerulitis
¾Focal
Wegener’s Granulomatosis: Gross

Pathology [Figures 2 and 3]
x Necrotic nodules
¾With and without cavitation
x Parenchymal consolidation
x Massive hemorrhage
x Airway narrowing
Leavitt, ARRD 1986.
Angiitis and Granulomatosis: Figure 2

Differential Multiple Vessel Associated
Solid and cavitary
Nodules [Figure 1]
nodules often
x Metastatic disease coexist in patients
¾Squamous with Wegener’s
x Multifocal infection granulomatosis.
¾Fungus, TB, bacteria
x Septic emboli
x Multiple pulmonary infarcts
x Langerhans Cell Histiocytosis
x Rheumatoid nodules
Angiitis and Granulomatosis 72 Chest Radiology

Pulmonary
x Most commonly affected (94%)
x Multiple bilateral nodules or masses
x Cavitation common (30-50%)
x Occasionally solitary mass or nodule
¾~"
+
¾All patients progress
x Less common
Figure 3 ¾Diffuse alveolar hemorrhage
x Pleural lesion and effusions are rare
Airway
narrowing is Renal
a common x /

":
complication. x Segmental necrotizing glomerulonephritis
x
!

x Large vessel vasculitis
Wegener’s Granulomatosis: Other

Organ Involvement
x Skin (50%)
¾Symmetric papulonecrotic lesion of
Wegener’s Granulomatosis: extremities
Demographics x Eye and orbit (30%)
x Rare ¾Scleritis, conjunctivitis, optic nerve
¾X<KKKKK

$ and retro-orbital mass
x 2nd-8th decades of life x Nervous system (30%)
x Average age-50 years ¾Mononeuritis multiplex
x Male = Female x Joints
¾$ !

Q_XV ¾Acute arthritis follows activity of
x May occur in children
Q¤
"V
Wegener’s Granulomatosis: Limited Wegener’s Granulomatosis: Airway

x Involvement of lungs alone Involvement [Figures 4 and 5]
x Clinical sparing x Endobronchial abnormalities
¾Kidneys ¾59% bronchoscopy
¾Upper respiratory tract x Subglottic stenosis
x Biopsy positive x Tracheobronchitis
¾When clinically normal ¾Ulcerating
x Better prognosis x Tracheal or bronchial stenosis
x Often multifocal
Wegener’s Granulomatosis: Clinical ¾Variable length of involvement
Presentation x CT key for evaluation
x Classic triad ¾CXR often normal
¾Sinusitis
¾Pulmonary symptoms
¾
+
x Variable onset and course
x Chronic URI symptoms
¾May persist for years before
pulmonary disease
x Overwhelming vasculitis
¾Diffuse
Figure 4
Upper Airway Focal airway

x Chronic nasal obstruction narrowing is
¾Chronic discharge a common
x Destruction of cartilaginous nasal septum complication
x “Saddle nose deformity” in Wegener’s.
x Laryngeal involvement
¾Subglottic stricture
x Eustachian tube obstruction
x Otitis media
x Cochlear nerve vasculitis
Chest Radiology 73 Angiitis and Granulomatosis

Evolution with Treatment [Figures 7 and 8]
Figure 7
Nodules
Figure 5 in varying
stages.
Collapse due
to airway
narrowing in
Wegener’s.
Wegener’s Granulomatosis:
Radiography
x Earliest lesions
¾Bilateral reticulo-nodular opacities
x Multifocal nodules
¾Bilateral
¾5mm-10cm
x Sharply marginated
Figure 8
x Cavitation 20-50%
x Evolution

"
¾Thick walls to thin walled cysts with
treatment Wegener’s Granulomatosis: Computed
x Airspace consolidation Tomography [Figure 9]
x Feeding vessels
Changing Presentation ¾88%
x Cavitation
Necrosis and Hemorrhage [Figure 6]
¾Nodules greater than 2cm
x Subpleural location
¾Predominant
x CT “halo sign”
x Pleural based lesions
¾Mimic infarcts
x Reveals more nodules
Figure 9
Nodules with feeding

vessels are common
in vasculitis.
Figure 6
Massive necrosis and hemorrhage in Wegener’s.

Diffuse Pulmonary Hemorrhage: Wegener’s Granulomatosis: Laboratory
Capillaritis [Figure 10] x ANCA
x Common ¾Serum antineutrophil cytoplasmic
¾Microscopic polyangiitis autoantibody
¾Wegener’s granulomatosis x c-ANCA cytoplasmic pattern
¾SLE ¾Proteinase 3
x Uncommon ¾==

=
:

¾Good pastures active disease

Anti-GBM ¾Positivity drops to 30% in remission
¾Collagen vascular x p-ANCA perinuclear pattern
¾Idiopathic pulmonary hemorrhage ¾Reacts with myeloperoxidase
¾Churg-Strauss syndrome ¾Positive in collagen vascular diseases
¾Behcet’s syndrome
¾IgA nephropathy Wegener’s Granulomatosis: Treatment
and Prognosis
x Universally fatal without treatment
x /!+
++:

q
disease
x Steroids and cyclophosphamide
¾Remission in 93%
x 5 year survival 90-95%
x Infectious complications
¾Relapse and drug toxicity require close
monitoring and follow-up imaging
x Relapse has different manifestations from
presentation
Churg-Strauss Syndrome: Allergic

x Described by Churg and Strauss
¾1951
Figure 10
x True systemic vasculitis
Left: Pulmonary hemorrhage in capillaritis. x Associated
¾Asthma
¾Allergic rhinitis
Microscopic Polyangiitis [Figure 11] ¾Blood eosinophilia
x Microscopic polyarteritis nodosa x Hypersensitivity response to inhaled
x Most common cause of pulmonary-renal antigen?
syndrome
x 5th decade Churg-Strauss Syndrome: Pathology
x Male > Female [Figure 12]
x Renal, musculoskeletal, pulmonary, GI x Necrotizing vasculitis
and cutaneous x |!

x “Allergic granulomas”
¾Extravascular
¾Eosinophils
¾Multinucleated giant cells
Figure 11
Microscopic polyangiitis.
Figure 12

Churg-Strauss Syndrome: Churg-Strauss Syndrome: Comparison
Demographics with Wegener’s
x 2nd-4th decades x CSS
x 28 years mean age of onset x High incidence of asthma
x Male = Female x High incidence of cardiac involvement
x Excellent response to steroids (47%)
x Less severe renal and sinus disease
Churg-Strauss Syndrome: Background x Associated with P-ANCA
x Late onset asthma
¾100% Churg-Strauss Syndrome: Therapy and
x Precedes CSS by weeks to years (30) Prognosis
x Severe rhinitis and sinusitis x Prognosis relates to early diagnosis and
¾70% therapy
x High dose steroids usually effective
Churg-Strauss Syndrome: Prodromal x Cyclophosphamide in resistant cases
Stage x Therapy stopped after 6-12 months of
x '
+

*!
! remission
x Blood eosinophilia
x Elevated IgE Necrotizing Sarcoid Granulomatosis -
x ¤
!
+ How is This Related to Sarcoidosis?
x Progressive asthma, sinus pain, x A distinct entity?
myocardial involvement ¾Katzenstein
x ]+

x Some reported cases are undiagnosed
x Abdominal pain infections
¾Diarrhea and eosinophilic peritonitis x Those with extrapulmonary involvement
x Myalgias and neuritis ¾Sarcoidosis
Churg-Strauss Syndrome: Vasculitic Necrotizing Sarcoid Granulomatosis:

Stage Demographics
x Increasingly severe and widespread x 3rd to 7th decades
symptoms x Mean age 49 years
x Lung x
¾Eosinophilic consolidation, miliary to ¾J&J<
2 cm nodules (without cavitation), and
diffuse hemorrhage Necrotizing Sarcoid Granulomatosis:
x Cardiac Pathology
¾Coronary vasculitis and eosinophilic x Noncaseating granulomas
myocarditis (50% of mortality) ¾Similar to sarcoidosis
x GI x Vasculitis
¾Ulcerations, perforations and ¾Pulmonary arteries
peritonitis ¾Pulmonary veins
¾Found in areas away from
Churg-Strauss Syndrome: Computed parenchymal granulomas
Tomography x Coagulative necrosis
x !
¾Widespread
¾Predominantly peripheral 59% ¾Main distinction from sarcoidosis
¾Effusions
x Nodules Necrotizing Sarcoid Granulomatosis:
¾12% Clinical Presentation
x Bronchial thickening x 100% lung involvement
x Dilatation x Cough most common symptom
¾12% x Chest pain, fever and dyspnea
x Interlobular septal thickening x Weight loss and fatigue
¾6% x May be asymptomatic
Worthy, AJR 1998. ¾15-40%
x Rare extrapulmonary involvement
Johkoh, Radiology 2000. ¾13%
x Aspergillus antigens in some patients
Koss et al Human Pathology 1980.

Necrotizing Sarcoid Granulomatosis:
Imaging [Figure 13]
x Hilar adenopathy
¾Variable
¾Up to 79%
x Nodules
¾Cavitation is common
¾Subpleural
¾Perivascular
x Parenchymal opacities
¾Same distribution
Figure 14
LYG is a large B-cell lymphoma.
Lymphomatoid Granulomatosis: Clinical

Presentation
x Lung involvement
¾100%
¾Cough and dyspnea
x Skin
¾39-53%
¾Nodules, ulcers and rash
x CNS
¾37-53%
Figure 13 x Renal
¾32-40%
Typical nodules in NSG.
x Malaise and weight loss
¾35%
Necrotizing Sarcoid Granulomatosis:
Prognosis and Therapy Lymphomatoid Granulomatosis:
x May require no therapy Imaging [Figure 15]
x Prompt response to steroids x Nodules
x No reported deaths ¾80%
¾Multiple
Lymphomatoid Granulomatosis: Etiology ¾Bilateral (80%)
and Demographics [Figure 14] x Mid and lower lobes
x Majority of cases are B-cell lymphomas x Cavitation
x Epstein-Barr virus ¾20%
x Reactive small T-cells x Large masses
¾
+
¾Correspond to infarcts
x Malignant B cells x Diffuse reticulonodular opacities
x Age range x Hilar adenopathy
¾7-85 years ¾25%
x Mean age of onset
¾48 years
x
QJ<V
Lymphomatoid Granulomatosis:
Pathology
x
Figure 15
¾Mixed cell population
Atypical lymphocytes, plasma cells, Peripheral
histiocytes opacities in
x Vascular invasion LYG.
x Vascular destruction
x Necrosis
¾Peribronchovascular
¾Peripheral

Lymphomatoid Granulomatosis: Bronchocentric Granulomatosis:
Treatment and Prognosis Treatment and Prognosis
x Mortality rate x Asthmatics respond to steroids
¾53-90% x Some cases remit without treatment
x Long term remissions reported x Must rule out treatable infection and
¾Cyclophosphamide and steroids Wegener’s granulomatosis
x All who fail therapy proceed to develop
lymphoma
¾12-47%
Bronchocentric Granulomatosis: Clinical

and Demographics - Asthmatics
x Average age 22 years
x Tissue manifestation of ABPA
x Dyspnea, cough, fever, malaise and
hemoptysis Figure 16
x Peripheral and tissue eosinophilia
x
"
Mucoid
impaction in
Bronchocentric Granulomatosis: Clinical patients with
BCG.
and D
Asthmatics
x Average age 50 years
x Males = Females
x Fungal infections
¾Histo, blastomyces, aspergillus
x Mycobacterial infections
x Rheumatoid arthritis
x Wegener’s granulomatosis
x Idiopathic
Bronchocentric Granulomatosis:
Pathology
x

x Early invasion of mucosa
¾Histiocytes
¾Eosinophils
Asthmatics
¾Neutrophils
¾Non-asthmatics
x Secondary involvement of adjacent
arteries
x Granulomatous destruction
¾Bronchial walls
x Bronchopneumonia
¾Distal to affected airways
Figure 17
Bronchocentric Granulomatosis: ABPA may be clinically unsuspected.
Imaging [Figure 16]
x Most often unilateral
¾75% Angiitis and Granulomatosis:
x Multiple or solitary nodules Differential - Multiple Vessel Associated
x Parenchymal consolidation Nodules
¾Upper lobe predominance x Metastatic disease
x

+
[ ¾Squamous
¾Bronchiectasis x Multifocal infection
¾Mucoid impaction ¾Fungus, TB, bacteria
x Septic emboli
BCG and Tuberculosis x Multiple pulmonary infarcts
x Langerhans’ cell histiocytosis
BCG and Aspergillus [Figure 17]
x Rheumatoid nodules
BCG?

Fungal Infection? “Until S Causes are Found ………
We Must Devise Syndromes”
Angiitis and Granulomatosis: Conclusion x Etiology
x Wegener’s granulomatosis x Prognosis
x Churg-Strauss syndrome x Therapy
¾Allergic granulomatosis
x Necrotizing sarcoid granulomatosis
x Bronchocentric granulomatosis
x Lymphomatoid granulomatosis
References
1. /!

!

&
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+
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Publishers, 1995;401-35.
2. }
}
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<=`_@`ZQV`XX`X&
3. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest
<==`@<KZQJVXJKX&
4. ]*
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5. /:
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+
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+
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6. Travis WD. Pathology of pulmonary granulomatous vasculitis. Sarcoidosis Vasc and Diffuse Lung Dis
<==@<X<_J^&
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12. Daum TE, Specks U, Colby TV, et al. Tracheobronchial involvement in Wegeners granulomatosis. Am J

<==`@<`<`JJ&
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17. Kornblut AD, Wolff SM, DeFries HO, Fauci AS. Symposium on granulomatous disorders of the head and

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<=ZJ@<`QXV^XZX&
18. Allen NB, Bressler PB. Diagnosis and treatment of the systemic and cutaneous necrotizinq vasculitis
&

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<==^@Z<Q<VJ_X`=&
19. Travis WD, Hoffman CS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s

:*
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Saunders Company, 1994; 411-9.

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25. Lee SJ, Berry GJ, Husari AW. Wegener’s granulomatosis presenting as right middle lobe obstruction. Chest
<==X@<KXQ`V<JX_&
26. Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary
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29. Maguire R, Fauci AS, Doppman JL, Wolff SM. Unusual radiographic features of Wegeners granulomatosis.
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30. Maskell SF, Lockwood CM, Flower CDR. Computed tomography of the lung in Wegeners granulomatosis.

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31. Papiris SA, Manoussakis MN, Drosos AA, Kontogiannis D, Constantopoulos SH, Moutsopoulos HM. Imaging
+
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32. Jaspan T, Davison AM, Walker WC. Spontaneous pneumothorax in Wegener’s granulomatosis. Thorax
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33. Crotz W, Mundinger A, Würtemberger C, Peter HH, Schollmeyer R. Radiographic course of pulmonary
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36. Erzurum SC, Underwood CA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest
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37. Primack SL, Hartman TE, Lee KS, Muller NL. Pulmonary nodules and the CT halo sign. Radiol 1994;
<=K`<X`&
38. Connolly S, Manson D, Eberhard A, Laxer RM, Smith C. CT appearance of pulmonary vasculitis in children.
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39. Foo SS, Weisbrod CL, Herman SJ, Chamberlain DW. Wegener granulomatosis presenting on CT with

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40. Stokes TC, McCann BC, Rees RT, Sims EH, Harrison BDW. Acute fulminating intrapulmonary haemorrhage

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41. Dugowson CE, Aitken ML. Unusual presentation of recurrent Wegeners granulomatosis. Chest
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42. Erzurum SC, Underwood CA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest
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43. Amundson DF. Cavitary pulmonary cryptococcosis complicating Churg¬-Strauss vasculitis. Southern Med J
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50. Dee PM, Arora NS, Innes DJ. The pulmonary manifestations of lymphomatoid granulomatosis. Radiol
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54. Hicken R Dobie JC, Frew E. The radiology of lymphomatoid granulomatosis in the lung. Clin Radiol
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Tuberculosis
Tuberculosis Mycobacteria
x Leading cause of death from infectious x Tuberculosis complex
disease ¾M. tuberculosis, M. bovis, M. africnum,
x Z<K

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M. microti
x JX

! x M. tuberculosis and M. bovis
x <X
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countries x Person-to-person transmission
x 80% 15-59 years of age
x Highest incidence M. tuberculosis: Pathologic Features
¾$!

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70% of adults x Virulence related to cell wall
¾No endotoxin or enzymes
Tuberculosis: History x Caseous necrosis
x Ancient disease x Caseating granuloma
x <ZZJ

! ¾Central caseous necrosis
¾Isolation of M. tuberculosis ¾Rim of histiocytes, giant cells
x <=__

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Tuberculosis Pre-Antibiotic Era
Tuberculosis: United States

x <=`X
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¾19,707 deaths
x <=Z`
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¾1,752 deaths
x <=Z<==J
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cases
¾HIV
¾Immigration
¾Congregate settings Figure 1
¾Deteriorating TB services The caseating
¾MDR-TB granuloma is
¾Decreasing TB research the hallmark
of TB. The
Tuberculosis: United States actively
x JKKJ
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growing
bacilli reside
¾`&J<KKKKK in the
¾43% decrease from 1992 macrophages
¾4-6% of population infected in the
15 million people periphery.
x 51% Foreign-born
¾Mexico, the Philippines, Vietnam, India
and China
x U.S.-born
¾African Americans 25% of all cases
¾Homeless, immunocompromised,
elderly
x Urban areas, coastal states, states
bordering Mexico
MMWR March 21,Vol 52, 2003.
Tuberculosis 84 Chestl Radiology

Tuberculosis: Pathogenesis Tuberculosis: Pathogenesis
x Inhaled bacteria [Figure 2] x Latent TB infection
¾Mid to lower lung zones ¾¤~
¾Ghon focus ¾No active signs of infection
x Survival of organisms [Figure 4]
¾

¾Superior segment lower lobe
Oxygen gradient
Figure 2 Lymphatic gradient
Even though Bucket handle rib motion
we tend to x Active TB infection [Figure 5]
think of TB as ¾5% within 2 years
an upper lobe ¾5-10% lifetime risk
disease, we ¾#'j
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J

inhale most
bacteria into ¾

the mid and underweight, silicosis, DM, renal

lower lung failure, gastrectomy, jejuoileal bypass,
zones. transplantation, head and neck cancer,
prolonged immunosuppressive therapy
Figure 4
Physiologic Gradients - A
The lymphatic
Physiologic Gradients - A gradient helps
explain the upper
Tuberculosis: Pathogenesis lobe distribution
x Inhaled bacteria of reactivation
tuberculosis.
¾Mid to lower lung zones
¾Ghon focus
x Regional lymph node spread [Figure 3]
¾Ranke complex
x ] !!

x Cell-mediated immunity
x Delayed hypersensitivity
¾Caseous necrosis
¾2-10 weeks
x Healing
Figure 3
Figure 5
/
tuberculosis The lymphatic
the ineffective gradient helps
macrophages explain the
carry bacteria upper lobe
to regional distribution of
lymph nodes reactivation
where they tuberculosis.
proliferate
and
disseminate.
Chest Radiology 85 Tuberculosis

Tuberculosis: Clinical Features ¾5% within 2 years
x Primary TB ¾5-10% lifetime risk
x Postprimary TB ¾#'j
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x Disseminated TB ¾

underweight, silicosis, DM, renal

Primary Tuberculosis: Clinical Features failure, gastrectomy, jejuoileal bypass,
x Asymptomatic 65% transplantation, head and neck cancer,
¾

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prolonged immunosuppressive therapy
x Progressive primary complex
¾Fever, cough, hemoptysis, weight loss Postprimary Tuberculosis: Pathogenesis
[Figures 7 and 8]
Primary Tuberculosis: Radiologic x Delayed hypersensitivity
Features x Liquifaction
x Lymphadenopathy [Figure 6 left] x Cavitation
¾Children 95%, young adults 43%, ¾Airway
elderly 10% ¾Vessel
¾Right paratracheal, hilar ¾Pleura
¾Peripheral enhancement, central low-
attenuation
x
:
[Figure 6 right]
¾Children
¾Anterior segments upper lobes Figure 7
¾Medial segment middle lobe
x Consolidation Postprimary
TB implies
¾Unifocal 75% reactivation of
¾Segmental, lobar, multifocal dormant bacilli.
¾Homogeneous, patchy, linear, nodular ?
x Pleural effusion by tissue
destruction.
¾Adults 38%, children 11%
Leung. Radiology 1999, Vol 210.
Figure 6
Left: Lymphadenopathy is hallmark of primary TB and

is more common in children.
Right: The lymph nodes which surround airways may
cause narrowing that results in atelectasis.
Figure 8
Cavitation and necrosis enables spread via the airway,
blood stream or pleura.
Postprimary Tuberculosis: Clinical
Features
x Reactivation Postprimary Tuberculosis: Radiologic
¾Fever, malaise, anorexia, weight loss, Features
anorexia, night sweats x Consolidation 50-70%
¾Dyspnea, cough, chest pain, x Cavitation 40-45%
hemoptysis x Nodules
x Active TB infection x Airways involvement

Postprimary Tuberculosis: Radiologic Postprimary Tuberculosis - Nodules
Features [Figures 11 and 12]
x Consolidation 50-70%
¾Heterogeneous, nodular, linear
¾Apical, posterior 85%, Superior
segments 14%
x Cavitation 40-45% [Figure 9]
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x Nodules
¾Tuberculoma
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lesions, upper lobes

¾Endobronchial spread [Figures 10 to 12]
Centrilobular, tree-in-bud, 100%
by CT
¾Hematogenous spread
Miliary 1-3mm, random
¾Bronchiectasis, bronchitis, airway
Figure 11
narrowing
Endobronchial spread leads to airways nodules.
Postprimary Tuberculosis - Cavitation
[Figures 9 and 10]
Figure 12
Figure 9
Endobronchial spread leads to airways nodules.
Cavitation mplies a large number of bacilli speeds the
progression of disease.
Thoracoplasty
Oleothorax
Figure 10
Endobronchial
spread leads to
airways nodules.
Chest Radiology 87 Tuberculosis

Plumbage [Figure 13] Hemoptysis - Bronchial Artery
Hemoptysis - Mycetoma
End-Stage Lung
Tuberculosis - Chest Wall
Tuberculosis - Pericardial
Tuberculosis: HIV/AIDS
x CD4 > 200
¾Well formed granulomas
¾Upper lobe cavities, consolidation and
nodules
x CD4 < 200
¾Poorly formed granulomas
¾Adenopathy, consolidation and miliary
Figure 13 disease
x CD4 < 60

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¾No hypersensitivity reaction
which was a complication of plumbage.
¾Organisms spread from GI tract
Postprimary Tuberculosis - Airways ¾Miliary Disease
Postprimary Tuberculosis: Assessment Tuberculosis and AIDS

of Activity Tuberculosis and AIDS - Low CD4
x

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+

x Inactive disease Tuberculosis: Diagnosis
¾Radiographic stability x Conventional methods
¾6mos ¾+

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x Negative cultures ¾
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¾Cavitation ¾~

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¾Consolidation x Radiometric methods
¾Ground glass x Polymerase chain reaction (PCR)
¾Centrilobular opacities x HPLC
Lee, et al. Chest 1996.
Summary
Tuberculosis: Complications x Primary TB
x End-stage disease ¾Consolidation
x Hemoptysis ¾Ipsilateral lymphadenopathy
¾Bronchial arteries in chronic cavities ¾Pleural effusion
¾Mycetoma x Postprimary TB
¾Rassmussen (pulmonary artery) ¾Consolidation
aneurysm ¾Cavitation
x Chest wall involvement ¾

x Pericardial involvement ¾Tracheobronchial spread
x Empyema
¾BPF, empyema necessitatis Tuberculosis Pre-Antibiotic Era
Reference
1. ]
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Bronchogenic Carcinoma
A 20th Century Disaster Lung Cancer Etiology - Cigarette
Smoking
Histological C
Tumors x 85-90% of lung cancer deaths
x World Health Organization x 25% of lung cancer in non-smokers
x Lung tumor editions attributed to passive smoke
¾1967 x

¾1981 ¾Number of cigarettes smoked
¾1999 ¾Depth of inhalation
¾2004 ¾Age at which smoking began
x Improve communication
x Consistent treatment Clinical Presentation
x Basis for comparative studies x Central tumors
x Prognosis ¾Cough
¾Wheezing
Changes in the 1999/2004 WHO ¾Hemoptysis
x Subclasses of adenomas ¾Pneumonia
x Preinvasive lesions x Extrapulmonary invasion
x Adenocarcinoma ¾Pain
x ~
+
[ ¾Pancoast syndrome
x Neuroendocrine tumors ¾SVC Syndrome
x Biphasic and pleomorphic tumors x Metastases
x Paraneoplastic syndromes
Incidence of Lung Cancer x Asymptomatic 10%
Gazdar, Semin Oncol 1988.
Paraneoplastic Syndromes
Histological Typing of Lung Tumors x Cachexia, malaise and fever
x Based on light microscopic criteria x Ectopic hormone production
x

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++
¾ACTH
x Graded by the most poorly differentiated ¾ADH
region ¾Hypercalcemia
x Histologic heterogeneity is the “rule” x Clubbing and HPO
x Thrombotic endocarditis
Histologic Typing of Lung Tumors ¾Non-bacterial
x

:&

x Migratory thrombophlebitis
x Stage determines prognosis in non-small
cell Lung Cancer and Clotting
x >95% of 1° lung tumors
¾Adeno Squamous Cell Carcinoma
¾Squamous x Terminology
¾Large cell ¾Squamous
¾Small cell Flattened cells
¾Combination of above ¾Epidermoid
Mimics differentiation of the
Lung Cancer Demographics epidermis
x Most common cancer in males world-wide x Rapid local growth
x Leading cause of cancer mortality in x Distant metastases later
women and men (United States) x Strong association
x Mortality rates in women began increasing ¾Cigarette smoking
in 1935 and surpassed breast cancer in
1987 Squamous Cell Carcinoma
x Pancoast syndrome
Age-Adjusted Cancer Death Rates - x Hyperparathyroidism
Males vs Females ¾Parathyroid-like substance
Cancer 49:1999. x Most common to present as
radiographically occult
Bronchogenic Carcinoma 90 Chest Radiology

Preinvasive Lesions: Squamous
Dysplasia
x Similar to cervical cancer
x Squamous metaplasia
x Progression
¾Dysplastic epithelium
Figure 2
x Carcinoma in situ
¾Full thickness dysplasia Squamous cell
x Precursor cancers are
predominantly central
¾Invasive squamous cell cancer and endobronchial as
Squamous Cell Carcinoma: Microscopic } /
tomogram.
Features
x Individual cell keratinization
¾Eosinophilia
x Keratin pearls
¾Well-differentiated tumors
x Intercellular bridges
Squamous Cell Carcinoma: Gross Atelectasis [Figures 3 to 5]

Features [Figure 1] x Peripheral nodule or mass
x Central lesion x 30%
¾Polypoid, endobronchial, exophytic
growth
x Central necrosis common
x Bronchial wall invasion
¾Common
¾Positive cytology
x Proximal growth
¾Along bronchial mucosa
Figure 3
Figure 1
Atelectasis in an adult smoker is lung cancer until
The majority proven otherwise.
of squamous
cell cancers
are central
lesions.
Squamous Cell Carcinoma: Radiographic

Features [Figure 2]
x Hilar or perihilar mass
x Bronchial wall thickening
¾Often focal
x Consolidation
¾Must clear completely
x Atelectasis Figure 4
x Peripheral nodule or mass
¾30% Typical central squamous cell carcinoma.
x Cavitation
Chest Radiology 91 Bronchogenic Carcinoma

Small Cell Lung Cancer
x Small cell carcinoma
¾Pure histology
x Variant
¾Combined
x Elimination
¾Oat cell
¾Intermediate cell
WHO 2004.
Small Cell Lung Cancer: Microscopic

Features
x Small, uniform cells
x Scant cytoplasm
x Necrosis is common
¾Often extensive
Figure 5 x > 10 mitosis per 10 HPF
Golden’s “S” sign. ¾Average 60-70
x Neuroendocrine morphology
x Neuroendocrine markers
Cavitation [Figure 6]
¾75%
x Light microscopy diagnosis
WHO 2004.
Small Cell Lung Cancer: Gross Features

x Large
x Central mass (90%)
x Bronchial compression
x No endobronchial lesion
x Proximal growth
¾Along submucosa
x Extensive necrosis
x Hemorrhage
Small Cell Lung Cancer: Radiographic

x Hilar or perihilar mass
Figure 6
x Mediastinal adenopathy
Cavitation is most common in squamous cell cancer. x Primary tumor
¾Rarely evident
x Cavitation
¾Extremely rare
Pancoast Tumor: Superior Sulcus Tumor
x Characteristic pain
¾8th cervical
¾2nd thoracic trunk
x Horner’s syndrome
x Destruction of bone
x Hand muscle atrophy
Pancoast, JAMA 1992.
Small Cell Lung Cancer

x Rapid growth
x Considered metastatic at presentation
x Poorest survival
x Strongest association with cigarette
smoking
Figure 7
Small cell tends to spread along the
peribronchovascular lymphatics without endobronchial
invasion.

Large Cell Carcinoma [Figure 9]
Figure 8
Small cell most commonly presents as a mediastinal
mass. Figure 9
Large cell cancers are commonly necrotic but rarely

Small Cell Lung Cancer cavitate.
x Cushing syndrome
x SIADH Adenocarcinoma: Etiology
x Eaton Lambert x Cigarette smoke causatively linked to lung
x Most common cause cancer
¾SVC Syndrome ¾1950
¾Squamous cell 18X’s Adeno
Small Cell Lung Cancer: Therapy
¾$

x Response to chemotherapy and
radiotherapy Adenocarcinoma: Etiology [Figure 10]
x

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x /

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x ]

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x Adenocarcinoma most common
¾Peripheral
Large Cell Carcinoma
x Filtered low-yield cigarettes
x Rapid growth
¾Smaller particles
x Location
¾Reduced nicotine
¾Segmental
¾Greater depth of puff
¾Subsegmental
¾Increased number of puffs
x Early metastases
¾N-nitrosamines
x Poor prognosis
x Other factors-10%
x Strong association with cigarette smoking
¾Passive smoke
Large Cell Carcinoma: Microscopic ¾Particulates
¾Cooking practices
Features
x Large cells
x Prominent nucleoli
x Poorly differentiated
x Diagnosis of exclusion
x Neuroendocrine features
Large Cell Carcinoma: Gross Features Figure 10

x Large and bulky
¾Greater than 3 cm Adenocarinoma,
x Soft the most common
x Large areas of necrosis lung cancer is
predominantly a
Large Cell Carcinoma: Radiographic peripheral lesion.
Features
x Usually peripheral
x 70% of tumors
¾> 4 cm at presentation

Adenocarcinoma: Microscopic Features
x Glands
x Papillary structures
x Mucin
¾Intracellular
¾Extracellular
x Prominent nucleoli
x Moderate cytoplasm
x Desmoplastic reaction
x “Scar carcinoma”
¾Rare!
Adenocarcinoma: Radiographic Features

x Peripheral (75%)
x Solitary mass or nodule
x

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x !

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x Lobulated

/

x Borders malignancy.
¾'
¾
x Spiculated
x Obstructive pneumonitis (25%)
Spiculation and Retraction [Figure 11]
Figure 13
Air bronchograms are commonly seen in

adenocarcinomas.
Figure 11
Adenocarinomas are commonly spiculated peripheral

nodules.
Scar Carcinoma [Figure 12]
Necrosis
Air Bronchogram [Figure 13]
Slow Growth
Atypical Adenomatous Hyperplasia:
Preinvasive Lesion [Figure 14]
x Atypical cuboidal epithelium
¾Lining alveoli
¾Lining bronchioles
x Found in lung cancer resection specimens
Figure 14
x Probable precursor
¾BAC %

**~ /
¾Invasive adenocarcinoma differentiation form a small BAC may be problematic.
x Patchy ground glass **~

x 5mm or less disturbed.
Kitamura, AJCP 1999.

Atypical Adenomatous Hyperplasia Bronchioloalveolar Carcinoma:
(AAH) Radiographic Features [Figure 16]
x Solitary nodule
Bronchioloalveolar Carcinoma: ¾Excellent prognosis
Microscopic Features ¾Resection
x Lepidic growth pattern x Consolidation
x No evidence ¾May be multifocal
¾Stromal invasion x Ground glass
¾Vascular invasion x Multiple nodules
¾Pleural invasion x May cavitate?
x Diagnosis cannot be made on a small Noguchi, Cancer 1995.
biopsy
x Requires thorough sampling of resected
specimen
WHO, 1999.
Bronchioloalveolar Carcinoma:
Mucinous Type
x Alveolar spaces distended with mucin
x Aerogenous spread is common
x Multifocal consolidation Figure 16
BAC usually
Bronchioloalveolar Carcinoma: Non- presents as
Mucinous Type an area of
x Alveoli lined with consolidation.
¾Clara cells
¾Type II cells
x
:

¾Common
x Close association
¾AAH
Bronchioloalveolar Carcinoma: Gross

Features [Figure 15]
x Consolidation
¾Focal
¾Multifocal Bronchioloalveolar Carcinoma
x Architecture
¾Preserved BAC Recurrence
BAC vs Adenocarcinoma
BAC - Adenocarcinoma: CT, Histology

and Doubling Time
x Type A
x Ground glass
x Localized BAC
x Doubling time
¾
ZZK

¾
J<_Z

Aoki, et al. AJR 2000.

and Doubling Time
x Type B
x Ground glass
Figure 15 x Localized BAC
Right: BAC often presents as as area of consolidation. x Doubling time
¾
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¾
J<_Z


BAC - Adenocarcinoma: CT, Histology AAH [Figure 18]
and Doubling Time
x Type C
x Solid attenuation
x Focal ground glass
x Spiculation
x Pleural tag
x Localized BAC
¾:

+
x Doubling time
¾
_J<X_


and Doubling Time
x Type D Figure 18
x Solid attenuation only The precursor lesion to BAC is AAH.
x Spiculation
x Pleural tag
x Poorly differentiated adenocarcinoma BAC [Figure 19]
x Doubling time
¾
J`J

¾
<J__KJ

AdenoCa Appearance and Prognosis
Adenocarinoma [Figure 17]
Figure 19
BAC demonstrates lepidic growth and presents as an

area of ground glass and/or consolidation.
Adenocarinoma - BAC Prognosis

[Figure 20]
Figure 17
One form of adenocarcinoma begins as an invasive

process and presents with a solid nodule.
BAC
Figure 20
Survival decreases with increasing amount of

consolidation and less ground glass opacity.

References
1. Charloux A, Hedelin G, Dietemann A, Ifoundza T, Roeslin N, Pauli G, Quoix E. Prognostic value of histology

*!

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2. Charloux A, Ouoix E, Wolkove N, Small D, Pauli G, Kreisman H. The increasing incidence of lung

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3. Colby T, Koss M, Travis W. Tumors of the Lower Respiratory Tract, Atlas of Tumor Pathology. Third ed.
!
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6. Hardy J, Smith I, Cherryman G, Vincent M, Judson I, Perren T, Williams M. The value of computed
tomographic (CT) scan surveillance in the detection and management of brain metastases in patients with

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7.

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8. Kitamura H, Kameda Y, Ito T, Hayashi H. Atypical adenomatous hyperplasia of the lung. Implications for
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9. Ko YC, Lee CH, Chen MJ, Huang CC, Chang WY, Lin HJ, Wang HZ, Chang PY. Risk factors for primary lung

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15. Sone S, Takashima S, Li F, Yang Z, Honda T, Maruyama Y, Hasegawa M, Yamanda T, Kubo K, Hanamura
K, Asakura K. Mass screening for lung cancer with mobile spiral computed tomography scanner [see
&
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<==Z@X`<Q=<<<V<J_J`&
16. Travis W, Colby T, Shimasato Y, Brambilla E. Histological Typing of Lung and Pleural Tumors., International

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19. Travis WD, Rush W, Flieder DB, Falk E=R, Fleming MV, Gal AA, Koss MN. Survival analysis of 200

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20. Travis WD, Travis LB, Devesa SS. Lung cancer [published erratum appears in Cancer 1995 Jun
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Chest Seminar 1
To Be Posted
Chest Seminar 1 100 Chest Radiology

Chest Seminar 2
To Be Posted
Chest Radiology 105 Chest Seminar 2

Pulmonary Hypertension
Aletta Ann Frazier, MD

Key Points Normal Pulmonary Circulation
x

!

x Low pressure system, high capacitance
(arterial) from postcapillary (venous) ¾Recruitment and distension
pulmonary hypertension x <<K

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x Spectrum of idiopathic and secondary systemic circulation

conditions ¾Low vasomotor tone
x Vascular histopathology and secondary x Right ventricle expends minimal energy to

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perfuse the pulmonary vascular bed
radiology of pulmonary hypertension
Precapillary (Arterial) Pulmonary HTN
Precapillary Pulmonary Circulation x '

!

[Figure 1] x Right heart pressure overload develops

late
x Imaging reveals pulmonary HTN & clues
to etiology
x '#

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¾Mean PA pressure > 25 mm Hg at
rest (normal 10)
Cor Pulmonale… the Best Predictor of

Clinical Outcome
x PV regurgitation
x RV hypertrophy & enlargement
x TV regurgitation & RAE
x Dilated IVC, hepatic veins
Normal Heart vs. Cor Pulmonale

Figure 1
American College of Chest Physicians
Precapillary (arterial) circulation and vascular
anatomy. Arterial vessels accompany the
dichotomously branching airways of the lung. “Venice C

x PAH
x PH with left-sided heart dz
x PH with lung dz, hypoxemia
Postcapillary Pulmonary Circulation x PH due to thromboembolic dz
[Figure 2] x Miscellaneous
Simonneau G. et al. J Am Coll Cardiol. 2004 Jun
16;43 (12 Suppl S):5S-12S.
Pulmonary HTN: Precapillary Etiologies

x Idiopathic
x Secondary
¾Chronic thromboembolic disease
¾Sickle cell disease
¾Eisenmenger physiology
¾

¾Connective tissue disease
¾Chronic hypoxia (COPD, IPF)
Figure 2
Postcapillary (venous) circulation drains the capillary

beds of the alveoli. Veins and venules course back to
the left atrium within interlobular septa.
Pulmonary Hypertension 110 Chest Radiology

Pulmonary Arterial HTN: Histology
[Figure 3] Figure 5
x Medial hypertrophy CT criteria
x Intimal proliferation for enlarged
x Thrombosis main PA in
precapillary
x Arteritis pulmonary
hypertension:
transverse
diameter > 29
mm.
Figure 3
Figure 6
Photo- CT
micrograph manifestations
demonstrates of cor
a muscular pulmonale:
artery dilated RA and
(adjacent RV, thickened
to airway) anterior RV
narrowed wall, and
by medial Q
hypertrophy interventricular
and septum.
obstructed by
intravascular
thrombus.
Idiopathic Pulmonary Hypertension
x

_`
¥
QX<V
x 6% “familial”
¾Autosomal dominant, incomplete
penetrance
Precapillary HTN: Imaging x Associations
x Dilated central arteries ¾HIV infection
x Pruning of peripheral vessels ¾Appetite suppressants
x Mosaic perfusion ¾Cocaine abuse
x Cor pulmonale ¾Chronic liver disease
x PA atherosclerosis x Mean survival 2.8 years without treatment
Idiopathic Pulmonary Hypertension Atrial Septal Defect [Figures 7 to 9]
[Figures 4 to 6]
Figure 7
Longstanding uncorrected ASD with acquired
Figure 4
Eisenmenger physiology in 35 year old female.
Radiographic features of pulmonary hypertension:
enlarged main pulmonary artery, dilated central hilar
vessels, and peripheral oligemia.
Chest Radiology 111 Pulmonary Hypertension

Chronic Thromboembolic Disease
[Figures 11 to 13]
Figure 11
Broad-based
intravascular soft
tissue density
in the right
PA, combined
with multiple
bronchial
arterial collateral
vessels, suggests
CTEPH.
Figure 8
Vascular pruning pattern.
Figure 12
Chronic thromboembolic disease: mural-based soft

tissue masses and calcium in the lumen of right main
PA.
Figure 9
%

atherosclerotic plaques in the main PA.
Ventricular Septal Defect [Figure 10]
Figure 13

Q
+

Q
"
Figure 10
69 year old male with uncorrected VSD and Chronic Thromboembolic Disease
Eisenmenger syndrome. x 4% of cases acute PE; presents within 2
years
x Symptomatic with >60% vascular bed
Eisenmenger Physiology occlusion
x Congenital L-to-R shunt x 5-year survival rate <35%
¾VSD, ASD, PDA x j

! !

Q!

¾Endocardial Cushion Defect exclude IPH)

x Shunt reversal (R-to-L) follows sustained
elevation in PVR
x PAH irreversible & requires lung
transplantation

CHronic Thromboembolic Disease: CT Postcapillary Pulmonary Circulation
Features
x Enlarged central PA Pulmonary Hypertension: Postcapillary
x |
«

+
Etiologies
¤
x Idiopathic
x Abrupt cut-offs (pruning) ¾Pulmonary veno-occlusive disease
x Bronchial arteries (50% of cases) (PVOD)
x Mosaic perfusion ¾Pulmonary capillary hemangiomatosis
x Pleural tags (healed infarcts) (PCH)
x Secondary
Chronic Thromboembolic Disease: ¾ Mitral valve stenosis
Imaging ¾ Left ventricular failure
x CT Angio ¾

¾94-100% sensitivity, 96-98% ¾
]+

!
¾
j

:

¾More sensitive than PA angio for tumor

proximal disease
¾Multiplanar recons depict disease Pulmonary Venous Hypertension
extent ¾Acute or chronic onset
x MR Cine & Phase - contrast imaging ¾Elevated wedge pressures (with
¾Right heart function exceptions)
¾]
¤

*
: ¾PAH - secondary
¾Shunt vols from bronchial arts to ¾Venous dilatation
pulmonary veins ¾Venous “arterialization”
¾R-to-L shunt via patent foramen ovale ¾$

¾Ç Interlobular septa
“Recanalized” Chronic Thrombus ¾Subpleural thickening
¾Ground glass opacities
“Organizing” Chronic Thrombus ¾Pleural effusion
Pulmonary Thromboendarterectomy PCH/PVOD

x 15-30% CTEPH patients x Capillary proliferation & dilatation
x Thrombi to segmental or subsegmental x Venous medial hypertrophy & intimal
level proliferation
x '
«

: x Recanalized thrombus in veins & venules
x Operative mortality 8-23%
x Improved long term survival - 75% at six PVOD/PCH
years x

Q

J<V
x <X
+

Other Thromboembolic Materials… x Fatal within 3-5 years
x Associations
Talcosis ¾Chemotheraphy
x Chronic IV injection of crushed tablets ¾Prior viremia
x (Methadone, amphetamines) ¾HIV
x Thrombogenic pharmaceutical binding ¾? Toxic exposure

x Granulomas coalesce into birefringent
particles
Talcosis: Imaging
x EARLY
¾Diffuse micronodular opacities
x LATE
¾Fibrosis
¾High density perihilar masses
¾Emphysema

PVOD/PCH [Figure 14] Mitral Stenosis
x ~+

+

#
clinically Diagnostic Strategy for Pulmonary

x j

:
Hypertension Recommended Imaging
pulmonary edema Studies by ACCP
x CT clues x CXR
¾Septal lines x Echocardiography with Doppler
¾}}

Q¤V x j
Q+
/|#
V
¾Normal left atrium ¾PA gram if positive (resectability)
ACCP Evidence-Based Clinical Practice Guide-
lines. Chest 2004;126:14S-34S.
Diagnostic Strategy for Pulmonary

Hypertension
x
!

¾CXR
Figure 14 ¾Echocardiography with Doppler
PVOD, a rare ¾j
cause of x $
postcapillary ¾!
//
pulmonary Precapillary vs. postcapillary origin

hypertension:
prominent
Clues to underlying etiology
interlobular ¾MRI
septa and
+
subpleural
!
+

edema. perfusion
Go with the Flow ….

x Pulmonary Arterial Hypertension
¾Enlarged central and hilar vessels
¾Pruned peripheral vessels
¾Mosaic perfusion
Mediastinal Fibrosis [Figure 15] ¾Cor pulmonale
¾PA atherosclerosis
x Pulmonary Venous Hypertension
¾Septal lines
¾Smooth pleural thickening
¾Ground glass opacities
¾Pleural effusion
Figure 15
Two coronal CT reconstructions show mediastinal

/+

at their entrance to left atrium, thereby creating
unilateral postcapillary pulmonary hypertension. (Note
that precapiillary hypertension is also evident: the
main PA is dilated and there is extrinsic compression

*/
{"
Mediastinal Fibrosis: Imaging

x Mediastinal contours abnormal
x Coarse calcium deposits
x Soft tissue replaces mediastinal fat
x Constriction, encasement of mediastinal
structures

References
1. Bergin CJ, Rios G, King MA, Belezzuoli E, Luna J, Auger WR. Accuracy of high-resolution CT in identifying
!

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&
\

\

<==@<<X^<^&
2. [
$
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/
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$

~

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3. [
\/
$!
~
]
]&

:

!

!

&
#

+&

<=@XXZJ^<&
4. [

j
&

!
|:
+

!

+
!
&

~
!
<==@X<_J&
5. Jones AT, Hansell DM, Evans TW. Quantifying pulmonary perfusion in primary pulmonary hypertension

! &
|

\
JKK_@JXJKJ^&
6.

[
\&
!
!

!
/
&
\

\

<==Z@<^K=``K&
7. *
\&

!

!&

JKKK@^`J`XK&
8. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial
!

:

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!
JKK_@<J<_$X_$&
9.
\~
}:
]&
/

::
&
\

/
<=Z_@Z^`Z<&
10.
$

$&

/

+
!

!
!

+

&
\
/!
'
<===@<_J^KZ&
11. Primack SL, Muller NL, Mayo JR, Remy-Jardin M, Remy J. Pulmonary parenchymal abnormalities of
:

! !
/
&
!
<==_@<_^X=_&
12.

#q
|
[
\

&

!

:*&
!
<=Z=@==K`J^&
13. Remy-Jardin M, Remy J, Louvegny S, Artaud D, Deschildre F, Duhamel A. Airway changes in chronic

/

XX
&

<==^@JKXX``K&
14.

$
#

&

!
/
+
!
!

::
&
\

\

JKK_@<ZX`^K&
15. Schoepf UJ, Costello P. Multidetector-row CT imaging of pulmonary embolism. Semin Roentgenol
JKKX@XZ<K<_&
16. $!
~
$*
$\
#
/|&

+

/

+

*!

!
+
++
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17. $
}

&

+

! &
\

JKK_
\
<@_X
Q<J
$
$V
`$<J$&
18. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for
predicting pulmonary hypertension in patients with parenchymal lung disease. Medical College of

]
/
}&
!
<==Z@<<X<J`K&
19. Worthy SA, Muller NL, Hartman TE, Swensen SJ, Padley SP, Hansell DM. Mosaic attenuation pattern on
!
/

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!

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:

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:

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<==^@JK`_`^K&

Pulmonary Metastases
Aletta Ann Frazier, MD

Key Points x Venous drainage via liver
x The pathogenesis of pulmonary ¾Colon
metastases is complex ¾Pancreas
x The spectrum of radiological ¾Stomach
+

!*
+
x Venous drainage via bone
spread ¾Prostate
x Many extrathoracic malignancies produce x Dual venous drainage (simultaneous
characteristic radiologic patterns of seeding)
pulmonary metastases ¾Kidney, Bladder, Ureters
¾Uterus, Cervix
Metastatic Disease to the Lung ¾Anus, Rectum
x Most common lung neoplasm x Complex venous (and lymphatic) drainage
x '
JK``
+

+
¾Breast
extrathoracic malignancy
x Lung is the ONLY site of metastatic Pathogenesis of Hematogenous
disease in 15-25% of these patients Metastases [Figure 1]
x Tumor cells penetrate draining venules
Metastatic Disease to the Lung: Routes x Enter systemic venous circulation
of Spread x Filtered by pulmonary arterial circulation
x Pulmonary & bronchial arteries
x Pulmonary & pleural lymphatics
x Thoracic duct
x Airways
x Transdiaphragmatic lymphatics
What are the Most Likely Primary

Extrathoracic Malignancies?
x Breast
x Colon
x Uterus
x Kidney
x Prostate
x Oropharynx
x Stomach
x Pancreas
Which Malignancies are the Most Highly

Predisposed?
x Choriocarcinoma
x Osteosarcoma
Figure 1
x Testicular tumors
x Melanoma Hematogenous metastases arise from tumor cells
x Ewing’s sarcoma which penetrate vessels and lymphatics at the primary
x Thyroid carcinoma site, and are transported to the right heart via the
x Kaposi’s sarcoma systemic venous circulation.
Principle: “Generalizing Sites”

x Certain tumors seed the lung directly,
!

:
!

organ (bone or liver)

x Systemic venous drainage directly to lung
¾Melanoma
¾Sarcomas
¾Choriocarcinoma
¾Thyroid
¾Kidney, Testes, Adrenal Gland
¾Oropharynx
Pulmonary Metastases 118 Chest Radiology

Pathogenesis of Hematogenous Parenchymal Nodular Metastases:
Metastases [Figure 2] HRCT-Pathologic Correlation: Nodules
x Adherence, extravasation in distal <1 cm [Figure 3]
arterioles x Peribronchovascular (12%)
x Expansile growth in interstitium and x Periseptal (28%)
alveoli x Intermediate (68%)
x Vascularization x Angiocentric (76%)
¾Pulmonary arteries ¾Directly-centered on feeding vessel
¾Bronchial circulation (18%)
¾Transpleural collaterals ¾Eccentric to feeding vessel (58%)
Hirakata, et al. AJR 1993;161:37-43.
Figure 3
Secondary
pulmonary lobule:
hematogenous
metastases may
be angiocentric
but are random
with respect to
the secondary
pulmonary lobular
architecture.
Figure 2
Bloodborne tumor cells arrest in distal arterioles
Parenchymal Nodules: Imaging
of the pulmonary circulation, extravasate into the Features: Chest CT [Figure 4]
interstitium, and establish nodules by expansile x Multiple
growth. x Peripheral, basilar
x Variable size
x “Random” - eccentrically located between
Parenchymal Nodules: Histology BVB & interlobular septa
x x Occasionally - angiocentric
x Homogeneous cell population x Less commonly - “cannonball” or miliary
x Adjacent to arteries and arterioles x Rarely
x Alveolar septa compressed or obliterated ¾Cavitary
¾
Nodular Metastases ¾Solitary
x Rounded, coalescent or multilobulated ¾Ground glass halo (hemorrhagic)
x Multiple Angiosarcoma
x Peripheral, basilar Choriocarcinoma
x Variable size Post therapy
x Mixed areas of viability, necrosis,
hemorrhage Figure 4
Parenchymal Nodules: Multidetector Metastatic colon

carcinoma in
Chest CT middle aged
x High sensitivity male: variable-
¾95% for nodules >1cm sized nodules
¾91% for nodules .5-1cm are random,
peripheral,
x ]*

QK

_K`
&&
V occasionally
¾Intrapulmonary lymph nodes angiocentric in
¾Granulomatous diseases location.
Sarcoidosis
Silicosis
¾Amyloidosis
¾Infection
Chest Radiology 119 Pulmonary Metastases

“Cannonball” Metastases [Figures 5 to 7] Figure 7
x Colorectal carcinoma
Cannonball
x Renal cell carcinoma metastases in
x Sarcomas a young adult
x Melanoma male with a
soft tissue
sarcoma
(scout; axial
lung and
mediastinal
CT images).
Micronodular (Miliary) Metastases

Figure 5 [Figure 8]
x Malignancies
Cannonball ¾Thyroid CA (papillary)
metastases
(gross
¾Choriocarcinoma
lung) in x Opacities may persist post-treatment
young adult (“sterile”)
male with x DDX
soft tissue ¾Miliary tuberculosis
sarcoma.
¾Viral pneumonia
¾Sarcoidosis
Pulmonary Metastases: Unusual

Manifestations
x :

nodules
x Lymphangitic carcinomatosis
x Tumor thromboembolism
x Endobronchial metastases
x Pleural-based metastases
Figure 6 Figure 8
Micronodular metastases in middle aged female with
Cannonball thyroid cancer.
metastases in
a young adult
male with a
soft tissue
sarcoma
(scout; axial
lung and
mediastinal
CT images).

Cavitation in Metastases [Figure 9] Solitary Metastasis vs. Lung Primary
x Incidence 4% (vs. 9% of lung primaries) x “The likelihood of a primary lung
x Malignancies cancer versus a metastasis depends
¾Squamous cell CA (head and neck; on the histologic characteristics of the
cervix) - 69% extrapulmonary neoplasm and the
¾Adeno CA (colon, breast) - 31% patient’s smoking history.”*
¾Sarcomas (bone) - spontaneous ptx Quint, et al. Radiology 2000;217:257-61.
x Wall thickness NOT indicative of benignity
x DDX Solitary Metastasis vs. Lung Primary
¾Septic emboli x A SPN is more likely to be bronchogenic
¾Vasculitis CA than a solitary met if the patient has
¾Collagen vascular disease
+
¾Head and neck
¾Bladder
¾Esophagus
Figure 9
¾Breast
Cavitary ¾Cervix
metastases
¾Bile Ducts
in elderly
male with ¾Ovary
oropharyngeal ¾Prostate
cancer. ¾Stomach
x The incidence is fairly equal in patients
*!

+
C

Metastases [Figure 10]
¾Kidney
x Malignancies
¾Colon
¾Osteo-, chondro- and synovial
¾Adrenal gland
sarcomas
¾Uterus
¾

¾Salivary or parotid gland

(ovary, thyroid, GI)
¾Thyroid gland
x Post-chemotherapy or post-radiation
x $

x Variable content
x Melanoma
¾Osteoid matrix
¾Sarcoma (soft tissue, bone)
¾~ !

¾Testicular carcinoma
¾Psammoma body formation
Parenchymal Nodules: Assessing
Figure 10
Therapeutic Response
x Tumor doubling time
¾25% increase in tumor diameter
metastases separated by time (Tdt)
in middle- ¾Nodule growth rates vary widely
aged female Histologic tumor cell type
with ovarian Within the same patient
cancer
Each nodule a different monoclonal
population with variable response
to chemo
x Document changes in several nodules in
Solitary Metastasis
the same patient
x
<JZ
+

x Impacts treatment strategy &
x 3-10% of all SPNs are solitary metastases
participation in clinical trials
x Variable margins
¾ Chojniak et al. Am J Clin Oncol 2003;26(4):374-
¾Multilobulated 77.
¾Spiculated
x Solitary Metastasis vs. Lung Primary
x “In patients with known primary
malignancies and single parenchymal
nodules, the overall incidence of second
primary lung carcinoma is greater than
that of solitary metastases.”*
Coppage, et al. J Thorac maging 1987;2(4):24-
37.

Parenchymal Nodules: Indications for Lymphangitic Carcinomatosis
Metastasectomy x Cords of tumor in lymphatics of the
x To cure, but only if interlobular septa and peribronchovascular
¾Complete resection possible interstitium
¾No extrathoracic metastases (EXCEPT x Edema and desmoplastic reaction
colon CA & liver mets) accentuate interstitial expansion,
¾Chemotherapy-insensitive tumors thickening
¾Multiple nodules NOT a
contraindication Lymphangitic Carcinomatosis: Imaging
x Prolongs 5-year survival Features - Chest Radiograph
¾

JK`K x Normal (50%)
¾
«
+

x Kerley B lines
40% x Reticulonodular opacities
¾Melanoma, renal cell, head & neck, x Subpleural edema
+
}
XK x Pleural effusion (30-50%)
¾/!
!
< x Hilar, mediastinal lymphadenopathy (20-
40%)
Yoneda, et al. Curr Opin Pulm Med 2000;6:356-
x [

63.
Lymphangitic Carcinomatosis Lymphangitic Carcinomatosis: Imaging

[Figure 11]
Features - Chest CT [Figure 12]
x Smooth or nodular thickening of
x Blood-borne tumor cells extravasate and
¾Bronchovascular bundles
invade lymphatic channels
¾Interlobular septa (Kerley’s lines;
x Tumor also enters lymphatics “retrograde”
polygonal arcades)
via mediastinal, hilar lymph nodes (25%)
¾]

Q
V
x Lymphatics expand with tumorlets and
x Ground glass opacities
edema
x Focal or unilateral distribution (50%)
¾Lung or breast CA
x Pleural effusion (30%)
x Lymphadenopathy (up to 50%)
Figure 11
Figure 12
Secondary
pulmonary lobule: Lymphangitic
lymphangitic carcinomatosis
carcinomatosis in a middle
produces smooth aged female
and nodular with breast
expansion of cancer.
bronchovascular
bundles sheaths
and interlobular
septa.
Tumor Embolism
x Lodges in distal arterioles (100-200
micron diameter)
x 26% cancer pts (at autopsy)
x ®<

Lymphangitic Carcinomatosis x Complications
x

ZK ¾Cor Pulmonale (PAH)
¾Lung ¾Lung infarction
¾Breast ¾Lung hemorrhage
¾Stomach x Parenchymal or lymphatic mets if
¾Pancreas extravasation
¾Prostate
¾Colon
x Incidence 6-55%
x $

!
x /

«
diffusing capacity
x ~
!
:

/[[

Tumor Embolism ¾Radiation
x Malignancies ¾Chemotherapy
¾Stomach ¾Surgery
¾Lung (esp. adenoCA) ¾Interventional bronchoscopy (stenting;
¾Breast laser or mechanical resection;
¾ChorioCA brachytherapy; photodynamic
¾Ovary therapy)
¾Prostate Chan, et al. Curr Opin Pulm Med 2003;9:301-8.
¾Liver
¾Kidney Endobronchial Metastases: Imaging
¾Lymphoma Features [Figure 14]
¾Right atrial myxoma x Intraluminal soft tissue mass
x Atelectasis or post-obstructive pneumonia
Tumor Embolism [Figure 13]
x Serpentine or nodular opacities (distal
x CXR mucoid impaction)
¾Typically normal x Hilar mass (if adjacent mediastinal
¾'+
*

invasion)
opacities x ~~¢
!

x CT
¾“Beading” of peripheral pulmonary
arteries
¾Mosaic perfusion
¾Wedge-shaped peripheral opacities
¾'+
":

lymphangitic carcinomatosis
Figure 14
Figure 13 Endobronchial metastases with left upper lobe collapse

(renal cell carcinoma).
Tumor thrombo-
embolism may Pleural Metastases [Figures 15 and 16]
produce beading
along peripheral
x Malignancies
bronchovascular ¾Lung
bundles, as well ¾Breast (50% of patients)
as pulmonary ¾Ovary
infarction ¾Stomach
(courtesy Marc V.
Gosselin, MD). ¾Lymphoma
x Via lymphangitic or vascular invasion
x Typically manifest as exudative pleural
effusion
¾Pleural nodules less common
Endobronchial Metastases x Scattered nodules on pleural surface
x Tumor arrives via bronchial arteries & x Visceral & parietal pleura typically both
peribronchial lymphatics involved
x Rarely, via airways (BAC) x Rind-like or sheet-like pattern
x 2-5% incidence in pts dying from x DDX of gross & rads
metastases ¾Mesothelioma
x Malignancies ¾
!"
¾Kidney
¾Colon, Rectum
¾Breast
Figure 15
¾Melanoma
¾Pancreas Large pleural
x
:
+

+

effusion and
65 months pleural-based
x
::
+
:
<`&`
nodule in patient
with breast
months cancer.
x Treatment options

Patterns of Metastatic Disease to the
Lung: Overview
x Parenchymal nodules
¾Well-circumscribed, random or
angiocentric, basilar>apical
¾
:

x Lymphangitic carcinomatosis
¾$

!

}}
¾Pleural effusion
¾Lymphadenopathy
x Tumor thromboembolism
¾Beading of peripheral arteries
¾Mosaic perfusion
¾Pleural-based opacity (infarction)
x Endobronchial nodule
¾Rounded defect in airway, or cut-off of
airway lumen
¾Post-obstructive atelectasis,
pneumonia, mucoid impaction
x Pleural-based metastases
¾Pleural effusion
¾Nodules on pleural surface
¾j

mesothelioma
Figure 16
Pleural rind-like metastases in elderly female with

NSCLC.

References
1. Chan AL, Yoneda KY, Allen RP, Albertson TE. Advances in the management of endobronchial lung
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2. !

&

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3. Coppage L, Shaw C, Curtis A. Metastatic disease to the chest in patients with extrathoracic malignancy. J
/!
'
<=Z^@JQ_VJ_X^&
4. David SD, Westcott J, Fleishon H, Gefter WB, Henschke CI, McLoud TC, Pugatch RD, Sostman HD, Tocino
I, White CD, Yankelevitz D, Bode FR. Screening for pulmonary metastases. American College of Radiology.

&

JKKK@J<`
$``J&
5. Davis S. CT evaluation for pulmonary metastases in patients with extrathoracic malignancy. Radiology
<==<@<ZK<<J&
6. Heffner JE, Milam MG. Sarcoid-like hilar and mediastinal lymphadenopathy in a patient with metastatic

&

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<@KQ^V<`_`^&
7. #

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#
\&

+

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!!

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<==X@<<X^_X&
8. Jungraithmayr W, Hasse J, Stoelben E. Completion pneumonectomy for lung metastases. EJSO
JKK_@XK<<<X^&
9. Kozuka T, Johkoh T, Hamada S, Maito H, Tomiyama N et al. Detection of pulmonary metastases with

*
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11. !
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12. Marglin S, Mortimer J, Castellino R. Radiologic investigation of thoracic metastases from unknown primary
&
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13.
|
¡!
|&
[

+

&
\
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<=Z^@JQ_V<`JX&
14.

/!!

* !

&

/!
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<==J@<ZJXX<`&
15. Parra ER et al. Pulmonary and mediastinal “sarcoidosis” following surgical resection of cancer. Pathol Res
Pract 2004;200(10);701-5.
16.
}

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/!
!
+

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<=ZK@
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17. !
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|:

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<==`@
<K^J=_$^$&
18. Quint L, Park C, Iannettoni M. Solitary pulmonary nodules in patients with extrapulmonary neoplasms.

JKKK@J<^J`^<&
19. $
\[
'
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20. Snyder BJ, Pugatch RD. Imaging characteristics of metastatic disease to the chest. Chest Surg Clin N Am
<==Z@ZQ<VJ=_Z&
21. Woodard PK, Dehdashti F, Putman CE. Radiologic diagnosis of extrathoracic metastases to the lung.

<==Z@<JQXV__<_&
22. Yoneda KY, Louie S, Shelton D. Approach to pulmonary metastases. Current Opinion in Pulmonary

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Differential Diagnosis of Mediastinal Masses
Melissa L. Rosado de Christenson, MD, FACR

Learning Objectives:
x /

!

!
mediastinal compartments
x To describe a practical approach to the
imaging diagnosis of mediastinal masses
x To list clinical and cross-sectional imaging
features that allow a focused differential
diagnosis of mediastinal masses
x To describe lesions with pathognomonic
imaging features
x To differentiate neoplastic from non-
neoplastic conditions with emphasis on
management (surgical vs. non-surgical)
Figure 2
Mediastinal Compartments [Figure 1]
Anatomic and surgical mediastinal compartments.
x Mediastinum – space between pleural
surfaces and lungs
x Bound by sternum and vertebrae
x From thoracic inlet to diaphragm
x Thymus, lymph nodes, heart, great
vessels, trachea, esophagus, nerves and
other soft tissues
x Arbitrary division into compartments – No
anatomic boundaries
Figure 1
The
mediastinum: Figure 3
space between Radiographic mediastinal compartments:
the pleural Felson / Fraser & Paré.
surfaces and
lungs. Mediastinal Masses - Mayo Clinic
(N=1,064)
x Thymoma 20%
x Cyst 20%
x Neurogenic 20%
Mediastinal Compartments [Figures 2 and 3] x Lymphoma 30%
x Anatomic – Superior, anterior, middle, x Teratoma
posterior x Granuloma
x Excludes paravertebral areas x Mediastinal goiter
x Surgical – Superior, anterior, middle,
posterior Wychulis et al. J Thorac Cardiovasc Surg 1971.
x Includes paravertebral areas
Approach to Mediastinal Masses
x Radiographic (Felson) – Anterior, middle,
x Clinical
posterior
¾~ !
Q
V
x Radiographic (Fraser, Müller, Colman,
Symptoms
Paré) – Anterior, middle-posterior,
x Radiography
paravertebral
¾Mediastinal compartment
Mediastinal Masses ¾Diffuse mediastinal enlargement vs.
x Patients are often asymptomatic focal mass
x 83% of asymptomatic masses are benign ¾

x 57% of symptomatic masses are x Cross-sectional imaging
malignant ¾$

!

x "
<X

normal structures
x "
<<K

: ¾!

$

Mediastinal Masses Chest Radiology

128
Mediastinal Masses Lymphoma
x Neoplasia x Non-Hodgkin lymphoma – 75% of all
¾
Q

++V cases
¾[

Q

x 50-70% of mediastinal lymphoma is
focal) Hodgkin disease
x Congenital cysts ¾15-21% is non-Hodgkin lymphoma
x Glandular enlargement (Thymus, Thyroid) x #

!

x Vascular lesions presentation

x #

|!
¾#
X^
!

x Miscellaneous conditions presentation

x /
!
!!
Malignant Neoplasia – Lung Cancer
x $

++

Lymphoma: Clinical Features
cancer x Hodgkin Disease
x Elderly smokers; males and females ¾Males = Females (NSHD, 2X as
x Cough, dyspnea, SVC syndrome, weight common in females)
loss ¾[

Jnd to 3rd and
x Mediastinal mass, lymphadenopathy, local > 5th decades
invasion ¾] !!
:
x Primary neoplasm may not be evident supraclavicular

x Non-surgical lesion ¾20-30%; fever, night sweats, weight
loss
Malignant Neoplasia - Metastases x Non-Hodgkin Lymphoma
[Figure 4] ¾Systemic disease with constitutional
x Known malignancy "&
!!

:
x Cell Types ¾] !
¯

!

¾Renal cell carcinoma adolescents

¾Testicular carcinoma ¾Diffuse large B cell – young adult
¾Head and neck cancer females
¾Breast carcinoma
¾Melanoma Lymphoma: Pathologic Features
¾Thyroid [Figures 5 and 6]
x Hodgkin Disease
¾

connective tissue (NS) Reed-Sternberg

cell
¾Lymphadenopathy, nodal coalescence,
primary thymic involvement, cystic
Figure 4
change, hemorrhage, necrosis
Metastatic renal ¾Local invasion
cell carcinoma: x Non-Hodgkin Lymphoma
diffuse bilateral ¾Lymphoblastic (precursor
mediastinal T-lymphoblastic) – lymphoblasts
enlargement.
¾Diffuse large B-cell (primary

!

[V@
large cells, vesicular nuclei, prominent

nucleoli
¾]

:

Malignant Neoplasia – Lymphoma invasive, necrosis

x #

#
lymphoma
x All age groups (young patients); Males
and females
x Palpable lymphadenopathy, constitutional Figure 5
symptoms
x ]

++

Hogdkin
x Prevascular, paratracheal disease:
Reed-
lymphadenopathy
Sternberg
¾Nodal coalescence, local invasion cell.
x Primary mediastinal lymphoma
x Non-surgical lesion
Chest Radiology 129 Mediastinal Masses

Figure 9
Non-Hodgkin
lymphoma:
nodal
coalescence
with focal low
attenuation
corresponding
Figure 6 to necrosis.
Hodgkin
disease:
nodal Non-Neoplastic Lymphadenopathy
coalescence. x Infection
¾

@

¾!

+

x Sarcoidosis
¾Bilateral symmetric hilar
lymphadenopathy
Lymphoma: Imaging Features
¾Typical pulmonary involvement
[Figures 7 to 9]
x Castleman disease
x Lobular diffuse bilateral mediastinal
¾|!

Q<KV
enlargement
¾#

!
Mediastinal Fibrosis
involvement in 85% x Granulomatous lymphadenopathy
Lymphadenopathy; prevascular, x Young patients with signs and symptoms
paratracheal of obstruction

Q!

¾Trachea, bronchi, esophagus, vessels
heterogeneous) x Mediastinal mass, circumscribed or locally
Cystic change ::

¤¤@
<

<

!
x Systemic antifungal agents, excision,
rare pre-therapy dilatation, bypass graft
¾#
:
x 30% mortality
paratracheal lymphadenopathy
Isolated involvement of other Castleman Disease
mediastinal lymph nodes x Angiofollicular or giant lymph node
x Local invasion hyperplasia
x Primary mediastinal lymphoma x Hyaline vascular type (> 90%) vs. plasma
cell variant
x Localized vs. systemic
x
+
Q

_<V

Figure 7 asymptomatic
x

!

Hodkin ¾Solitary mass
Disease:
bilateral ¾Dominant mass with lymphadenopathy
lobular ¾Multiple enlarged lymph nodes
mediastinal ¾|!

Q<KV
enlargement.
Secondary Neoplasia: Pearls
x Malignant
¾Diffuse bilateral mediastinal
enlargement
¾Lymphadenopathy (nodal coalescence)
Figure 8 ¾Local invasion
Hodgkin x Differential diagnosis
disease: ¾Advanced lung cancer - elderly
prevascular / smokers
paratracheal
lymphade- ¾Metastases - known malignancy (GU)
nopathy and ¾Lymphoma - young adult (wide age
left pleural range)
effusion.
Mediastinal Masses 130 Chest Radiology

Primary Neoplasia
x Thymus
¾Thymoma
¾Thymic malignancy
¾Thymolipoma
¾Germ cell neoplasm
x Neurogenic neoplasms
Thymoma Figure 11
x Epithelial neoplasm, most common
Thymoma:
primary thymic neoplasm
&
x Slow growth, “benign” behavior tumor lobules
x M = F; 70% in the 5th and 6th decades compart-
x Most patients asymptomatic mentalized by
x J`XK
*!

+

"
invasion
x
!

¾Myasthenia gravis
¾Pure red cell aplasia
¾Hypogammaglobulinemia
Thymoma and Myasthenia Gravis

x Myasthenia gravis (MG) – autoimmune
neurological disorder
x 85% of patients with MG have follicular
thymic hyperplasia
x 15% of patients with MG have thymoma
x Of all patients with thymoma, 30-50%
have MG
Figure 12
Thymoma: Pathologic Features
[Figures 10 to 13] Thymoma:
x Lymphocytes and epithelial cells in central
varying proportions hemorrhage
and necrosis.
x #
<===

Q!

lymphocyte-to-epithelial cell ratios)

x Types A, AB, B1, B2, B3
x Tumor lobules compartmentalized by

x /!
!
+
x Encapsulated vs. Invasive
x Spherical mass, variable size, lobular
contours, typically encapsulated
x Hemorrhage, necrosis, cystic change
(mural nodules)
x '::
!

documentation of capsular invasion

¾Local invasion, tumor implants,
metastases
Figure 13
Cystic lesion
Figure 10 with lobular
mural nodules.
Thymoma:
tumor lobules
compart-
mentalized by

"

Thymoma: Imaging Features Figure 17
[Figures 14 to 18]
x Anterior mediastinal mass; lobular, Cystic
thymoma:
unilateral, variable size spherical right
x Normal radiographs in 25% (occult thymic cystic
thymoma) mass with
x Focal, spherical, homogeneous or peripheral
heterogeneous curvilinear

¾Necrosis, cystic (mural nodules), and mural

Q
:

nodules.
peripheral)
x No lymphadenopathy
x Exclude local invasion of fat,
cardiovascular structures, lung
x Pleural implants (may cause diffuse Figure 18
pleural thickening) ++
thymoma:
direct invasion
of left brachio
cephalic vein.
Thymoma: Staging (Masaoka) /

Survival (10 Year)
I
|

invasion (86 – 100%)

Figure 14 II Microscopic invasion into surrounding fat,
Thymoma: unilateral, lobular, left anterior mediastinal mediastinal leura, microscopic capsular
mass. invasion (55 - 100%)
III Macroscopic invasion of adjacent organs;
pericardium, great vessels, lung (47 -
60%)
Ij

pericardial dissemination (0 -
Figure 15
11%)
Thymoma: Ij
] !

!

spherical soft
tissue mass Thymoma: Therapy / Prognosis
in left thymic x Encapsulated; complete excision
lobe.
¾Best prognosis
¾Occasional local recurrence, distant
metastases
x Postoperative radiation for invasive
thymoma to decrease local recurrence
x !!
+

recurrence after surgery and unresectable
lesions
Figure 16
Thymoma:
Thymic Malignancy: Carcinoid /
$ Carcinoma
large right x Rare malignant epithelial neoplasms
cardiophrenic x Symptomatic patients
angle mass x Poor prognosis
with extensive
low attenuation
corresponding
to necrosis.

Thymic Carcinoid
x Neuroendocrine neoplasm; atypical
carcinoid (necrosis, mitoses, invasion)
x
¥
@
X<@
*

(43 years)
x 50% functionally active
¾ACTH – Cushing syndrome (33 - 40%)
x MEN type 1 – (Wermer syndrome) (19 -
Figure 20
25%)
¾Hyperparathyroidism (90%), islet cell Thymolipoma:
tumor of pancreas (80%), pituitary thymic tissue
admixed
adenoma (65%) with mature
adipose
Thymic Carcinoma tissue.
x
¥
@
*

Q
5th decade)
x Several cell types identical to primary
lung cancer
¾

x WHO Type C thymoma
Thymic Carcinoid / Carcinoma Imaging

Features [Figure 19] Figure 21
x Large anterior mediastinal mass
Q
! V Thymolipoma:
x

anterior
mediastinal
(histology) mass with
x Crosses midline anatomic
x Lymphadenopathy connection to
x Local invasion, pleural or pericardial the thymus
and mixture
++

of fat and
x Carcinoid soft tissue
¾Octreotide imaging for occult lesions elements.
Q
¯

!
thymic neoplasms)
Thymolipoma: Imaging Features
x
+

Figure 19 mass
Thymic ¾Unilateral or bilateral, slow growth
carcinoid: x May conform to shape of structures
mediastinal ¾

mass with diaphragmatic elevation
adjacent
lymphaden- ¾Positional change in shape
opathy ¾Anatomic connection to the thymus
associated (pedicle)
with ACTH ¾"
+

+

production signal
and MEN 1.
Germ Cell Neoplasms
x Most common in the gonad
Thymolipoma x Extragonadal germ cell neoplasms;
x Rare benign thymic neoplasm midline locations, most commonly the
x M = F; wide age range (28 yrs.) mediastinum
x

`K x Postulated origin in multipotential
¾Symptoms with large tumors primitive germ cells “misplaced” during
embryogenenesis
Thymolipoma: Pathologic Features x

[Figures 20 and 21] x Teratoma (mature, immature [immature
x Encapsulated, soft, lobular, yellow

"
x Mature adipose tissue and thymic tissue

V
in variable proportions x Seminoma
x Non-seminomatous germ cell neoplasms

Mature Teratoma Mature Teratoma: Imaging Features
x 60-75% of mediastinal germ cell [Figure 24]
neoplasms x Unilateral anterior mediastinal mass
x Males = Females x $!

*
x Children and young adults (<40 years) x Multilocular cystic – 85%
x Often asymptomatic x

Z=

^
¤¤
x Symptoms of compression or rupture 53%

¾
:

<<
Mature Teratoma: Pathologic Features ¾$/]/¤¤

X=
[Figures 22 and 23] ¾$/]/

J_
x More than one embryonic germ cell layer ¾$/]

<`
¾|

¾

Figure 24
¾|
}'

mucus glands Mature

x Spherical, encapsulated, lobular borders teratoma:
unilateral,
x Multilocular or unilocular cystic mass anterior
¾Oily, sebaceous, gelatinous material mediastinal,
(lipid) multilocular
¾

#
!
cystic mass
with intrinsic
Q&

tissue, fat and
calcium.
Mature Teratoma: Therapy/Prognosis

x Complete excision is curative
Figure 22
x Excellent prognosis
¾
<KK
:
::
Mature
teratoma: Seminoma
cystic
neoplasm x 40% of malignant germ cell neoplasms of
with a single histology
ectodermal, x Caucasian males, third to fourth decades
mesodermal x Most patients are symptomatic
and x Round cells with sharp borders, clear
endodermal
components.

!
plasma cells, non-caseating epithelioid

granulomas
x Homogeneous soft tissue mass
x
!

chemotherapy
¾60-80% long-term survival
Seminoma: Imaging Features [Figure 25]

x Anterior mediastinal mass (bilateral
growth)
¾]

*

Figure 23
locally invasive
x /
Mature ¾Homogeneous soft tissue mass
teratoma:
¾Mimics nodal coalescence
multilocular
cystic tumor ¾Slight homogeneous contrast
with lipid-rich enhancement
cyst content. ¾

!
QZV
Figure 25
Seminoma: diffuse
homogeneous
anterior mediastinal
mass with mass
effect.

Non-Seminomatous Malignant Germ Cell mediastinal neurogenic neoplasm
Neoplasms [Figure 26] ¾$!++

x Yolk sack (endodermal sinus) tumor x

!
!!
x Embryonal carcinoma x Young adults; 3rd and 4th decade
x Choriocarcinoma x Most (65%) asymptomatic
x Mixed germ cell neoplasm x Symptoms and signs of compression
x Males, 90% symptomatic
¾Klinefelter syndrome (20%);
hematologic malignancy Figure 27
x Serology
Neurogenic
¾Alpha-fetoprotein (EST, EC) neoplasms
¾ß-human chorionic gonadotropin – ß may arise
HCG (choriocarcinoma) from
¾LDH (60%) tumor burden peripheral
nerves or
x Large, unencapsulated
sympathetic
x Hemorrhage, necrosis, “cyst” formation ganglia.
x Cisplatin-based chemotherapy, excision of
residual tumor
Figure 26
Non-
seminomatous
malignant germ
cell neoplasm:
large locally
invasive
heterogeneous
anterior
Figure 28
mediastinal
mass with
central Schwannoma:
necrosis. heterogeneous
spherical mass.
Non-Seminomatous GCN: Imaging

Features
x ]
*

mediastinal mass
¾Extends to both sides of midline
x Heterogeneous
¾Large areas of central low attenuation
¾Frond-like peripheral soft tissue
x Loss of tissue planes
¾Local invasion, lymphadenopathy Schwannoma/N ! " Imaging
Neurogenic Neoplasms [Figure 27] x $!
!
*
x 20% of primary mediastinal neoplasms paravertebral mass

(35% in children) x

Q`KV@

x 70–80% benign +

+

:@
x Peripheral nerves expanded neuroforamen

¾Schwannoma x # ! @
¾ ¾#
!@
¤¤
¾Malignant peripheral in 10%

¾Nerve sheath tumor ¾Intraspinal growth - 10%
x Sympathetic ganglia x
'
¯

::
¾Ganglioneuroma ¾T1 – Low-to-intermediate signal
Ganglioneuroblastoma ¾T2 – Foci of high signal
¾Neuroblastoma
Malignant Peripheral Nerve Sheath
Schwannoma/N ! [Figure 28] Tumor
x Schwannoma – Most common mediastinal x Most frequent in the paravertebral region
neurogenic neoplasm; x Rare among neurogenic neoplasms
¾Spherical, encapsulated x Large (> 5 cm) spherical mass
¾Cellular and less cellular areas (Antoni ¾Central low attenuation – necrosis
[V ¾
x
¯

¾May exhibit local invasion

Figure 31
Ganglioneuroma:
elongate
paravertebral mass.
Figure 29
Schwannoma: unilateral paravertebral spherical mass

with benign pressure erosion on posterior third rib.
Figure 32
Ganglioneuroma: elongate
paravertebral mass with
benign pressure erosion on

"
Figure 30
Schwannoma: neuroforaminal and intraspinal growth.
Peripheral Nerve Neoplasms: Therapy

and Prognosis
x Excision
x $!*
¾Excellent prognosis
x Malignant peripheral nerve sheath tumor Figure 33
¾$
¯
^`
:
:: Ganglioneuroma:
¾
¯
XK
:
elongate
survival paravertebral mass
with benign pressure
N ! #NF1) erosion on right

"
x Multiple neoplasms (including
ganglioneuroma)
x "+

x Vagus nerve, sympathetic chain, phrenic Ganglioneuroblastoma/Neuroblastoma
nerve x Infants and young children
x Diffuse enlargement of peripheral nerve x Asymptomatic; chest wall pain,
x Multiple masses along a nerve paraplegia, Horner syndrome, diarrhea,
hemothorax
Ganglioneuroma [Figure 31] x Elevation of urine catecholamines
x Children, adolescents, young adults ¾|:
+

j
¾Asymptomatic patients (screening)

x De novo; maturation of neuroblastoma x Neuroblastoma
x Benign paravertebral neoplasm ¾50% < 2 years
x Mature ganglion cells, Schwann cells, ¾90% < 5 years
:
¾May be congenital
x Encapsulated, elongate mass
¾} *
*!

+ Ganglioneuroblastoma/Neuroblastoma
Pathologic Features
Ganglioneuroma: Imaging Features x Adrenal - most common location
[Figures 32 and 33] ¾Paravertebral – second most common
x

:
location
x
x }

x Homogeneous or heterogeneous ganglion cells

¾

J` ¾ ++
x
#

x

#
/</J Wright pseudorosettes
¾

*! ¾ ++

Neuroblastoma: Imaging Features ¾Lymphadenopathy
[Figure 34] ¾Local invasion
x

:
mass Bronchogenic Cyst [Figure 35]

x !
:
+
¤¤

<K x Most common congenital cyst of the
x Osseous erosion mediastinum
x

*! x Abnormal ventral foregut bud
x Local soft tissue invasion x Failure to induce mesenchymal
development to lung parenchyma
x Mediastinum (85%), pericardium,
diaphragm, pleura, lung
Figure 35
Figure 34
Bronchogenic
=

+ cyst: typical
with intraspinal extension in a neonate. subcarinal
location.
Sympathetic Ganglia Tumors
x Ganglioneuroma
¾Excision is curative
x Ganglioneuroblastoma
¾Five-year survival near 90%
x Neuroblastoma
¾Five-year survival – 30%
¾
+:

*!

®
J
Bronchogenic Cyst: Clinical Features
years, mediastinal x Rare in infants, infrequent in children
¾Spontaneous maturation to x Young adults
ganglioneuroma x

x Symptomatic - chest pain, mass effect,
Paraganglioma obstruction, infection
x

x Excision, observation, drainage, sterile
¾:

!
alcohol ablation
paraganglia
¾Heart Bronchogenic Cyst: Pathologic Features
x Adults (average age 30 - 40 yrs.) [Figures 36 and 37]
¾
¥
@
J< x Respiratory epithelium
¾Asymptomatic; excess catecholamines x
[!

!
x
!
muscle
¾# ! x Closed foregut connection
x Marked contrast enhancement x Spherical, ovoid, unilocular
¾90% uptake of 1131 or 1123 MIBG x Thin wall
x
:

!!
Thoracic Meningocele serous, viscous

x Intrathoracic extrusion of meninges and
!

x
*!

^`
of cases
x
!
:

x Enlarged neuroforamen, pressure erosion,
Figure 36
sclerosis
x #

Bronchogenic cyst:
lined by respiratory
Primary Neoplasia - Pearls epithelium with
x Benign cartilage and smooth
¾Focal, unilateral mass muscle in wall.
¾No lymphadenopathy
¾No local invasion
x Malignant
¾++


Other Congenital Cysts
x Foregut Cysts
x Esophageal – within esophageal wall;
ectopic gastric mucosa
x Neuroenteric – associated spinal anomaly
x Pericardial – cardiophrenic angle,
Figure 37
*

@
Bronchogenic asymptomatic patients

cyst: thin-
walled ovoid Thymic Cyst [Figures 40 and 41]
unilocular x Uncommon (3% of mediastinal masses)
cyst. x Acquired vs. Congenital
x !

x Association with neoplasia, AIDS,
x ~++
:
!
syndrome (DILS)
x Epithelial lining and thymus in cyst wall
Bronchogenic Cyst: Imaging Features x
[Figures 38 and 39] x

x
!

mediastinal mass
x Near trachea, carina, stem bronchi
x /
¾Thin smooth wall (enhancement)

¾Water (40%) or soft tissue (43%)
attenuation
¾# !

enhancing content
Figure 40
x
¾T1 – variable (slightly hyperintense to Thymic cyst:
multilocular
muscle) cyst.
¾T2 – isointense or hyperintense to CSF
x /!*

@

:
Figure 41
Figure 38
Thymic cyst:
Bronchogenic cyst: subcarinal spherical mass with Multilocular
extension to the right and mass effect on bronchus anterior
intermedius. mediastinal
cyst with
enhancing
tissue septa.
Mediastinal Cysts - Pearls

x Focal, spherical or ovoid
Figure 39
x Unilocular (multilocular thymic)
Bronchogenic cyst: spherical subcarinal mass with x Thin-walled
water or soft tissue attenuation. x No mural nodules
x Foregut cysts along foregut-derived
structures

Thymic Hyperplasia
x Lymphoid hyperplasia (lymphofollicular

! V

follicles with germinal centers; may not

produce thymus enlargement
x Myasthenia gravis, hyperthyroidism,
lupus, scleroderma, rheumatoid arthritis,
cirrhosis Figure 43
x True hyperplasia - global increase in the
size and weight of the thymus Mediastinal
x Rebound hyperplasia - following goiter: large
chemotherapy (2 weeks to 14 months),
steroids or severe insult unilateral
mass with
x Thymic Hyperplasia extension into
x Anterior mediastinal widening the neck and
x Homogeneous soft tissue mass effect on
x Maximal thickness suprasternal
¾Under 20 years – 1.8 cm trachea.
¾Over 20 years – 1.3 cm
x Follicular thymic hyperplasia – normal or
mildly enlarged thymus
Mediastinal Goiter [Figure 42]

x 20% of cervical goiters
x
+

¾May produce symptoms by mass effect
x Adenomatous goiter; rarely malignancy or Figure 44
thyroiditis
x Fibrous capsule; nodules composed of Mediastinal
goiter:
thyroid follicles continuity
x #!

! between
cervical and
mediastinal
components,
high
attenuation
and

"
Parathyroid Adenoma
x |
!

Figure 42 of thymus (39%), mediastinum (2%),

Mediastinal goiter: thyroid follicles with iodine content intrathyroid (0.2-3.5%)
`{&$
`{" x Primary hyperparathyroidism post surgical
parathyroidectomy
Mediastinal Goiter: Imaging Features x MEN I
[Figures 43 and 44]
x '
x Unilateral anterior mediastinal mass ¾/==/JK<

(80%) /<JX/JK<
/==

$
¾Other compartments also affected, (mitochondria)

R>L ¾$

x

x /'

+

x Continuity with cervical thyroid
Glandular Enlargement - Pearls
x

x Anatomically related to normal gland

curvilinear
x Continuity with normal gland
x Cystic change
x Morphology and function similar to that of
x High attenuation
normal gland
¾Intense, sustained contrast
enhancement
Lymphangioma Lymphangioma: Imaging Features
x Benign mesenchymal mediastinal tumor [Figures 47 and 48]
x Proliferation of lymphatic vessels without x Anterosuperior mediastinum; other
communication with lymphatic tree compartments affected
x Developmental vs. neoplasm vs. x :" !
*
@
hamartoma mediastinal extension

x
+
x $!

*

x Mediastinal extension of cystic hygroma x
:

(10%), soft palpable mass; 90% x Multilocular, cystic, heterogeneous
diagnosed in infancy ¾Solid components, tissue septa
x

!

adult
Lymphangioma: Pathologic Features

[Figures 45 and 46]
x Spaces of variable size lined by
endothelial cells
x Soft, cystic mass
x Cystic hygroma – large vascular spaces
x Cavernous lymphangioma – small
vascular spaces
Figure 47
Lymphangioma: multilocular cystic mediastinal mass
with extension into the right axilla.
Figure 45
Lymphangioma:
interconnecting
endothelial
lined vascular
channels
containing
/ Q"
Figure 48
%/
+`{

?`{
multilocular cystic mediastinal mass.
Hemangioma [Figures 49 and 50]

x Rare vascular mediastinal tumor
¾Neoplasm vs. developmental
x Young patients; 75% < 35 yrs.
x @
<X<J
*!

+
compression
¾Rendu-Osler-Weber syndrome
x Communicating vascular spaces
Figure 46 ¾Endothelial lining, organized thrombi,
¤¤
!!
Lymphangioma: x Anterior mediastinal mass (also in other
multilocular compartments)
cystic x $!
*
¤¤
JZ
appearance due punctate, phleboliths

to distention and
enlargement
x Heterogeneous intense enhancement
of vascular
channels. Figure 49
Hemangioma:
anterior mediastinal
mass with intrinsic
phleboliths.

Figure 50 Figure 51
Hemangioma: Hiatus hernia:

intense herniation of
heterogeneous abdominal
enhancement. contents
through
esophageal
hiatus.
Vascular Lesion - Aneurysm

x Abnormal mediastinal contour contiguous
with vascular structures Herniations - Pearls
x Saccular aneurysms may resemble other x Intrathoracic extension of abdominal
primary mediastinal masses contents
x :
!
¾Bowel
x Contrast enhancement ¾Omental fat
x Continuity with vascular lumen x Esophageal hiatus
x Morgagni hernias
Vascular Lesion - Varices
x |! ! Miscellaneous – Achalasia
x Severe liver disease and portal x Absent peristalsis with incomplete
hypertension; Left gastric – portosystemic relaxation of lower esophageal sphincter
collaterals x
¯

+

x Visible on radiography in 10% myenteric plexus

x Middle-posterior-paravertebral cluster x Secondary – (pseudo achalasia) Chagas
of serpiginous vessels with intense disease and primary or secondary
enhancement malignancy at the GE junction
x |!

*!

:
Vascular Lesions - Pearls ¾Esophageal displacement to the right,
x
:&
: mass effect on mediastinum (posterior
x Lymphatic trachea), pulmonary consolidation
¾Multilocular cystic (aspiration)
¾Extramediastinal involvement
x Blood vessels Miscellaneous - EMH
¾'
!
x Extramedullary hematopoiesis
enhancement x Compensatory formation of blood
Pleboliths in hemangiomas elements outside osseous medulla
x Aneurysms x Hemolytic anemia
¾Focal vascular enlargement x Unilateral or bilateral paravertebral mass;
may exhibit internal fat attenuation
Herniations – Hiatus Hernia [Figure 51] x Adjacent medullary expansion
x Gastric herniation through enlarged
esophageal hiatus Miscellaneous – Acute Mediastinitis
x '

x Surgery, instrumentation with esophageal
Increased prevalence with increasing age perforation
x @
" x Ill patients with fever, chills and chest
x Retrocardiac mass, homogeneous, air- pain

x Focal or diffuse mediastinal widening,
x '
+

pneumomediastinum, pleural effusion,
hernia sac pneumothorax
x Abscess, abnormal mediastinal air,
Herniation - Morgagni extraluminal ingested contrast,
x Developmental defect in right obliteration of tissue planes
anteromedial hemidiaphragm
x

x Right cardiophrenic angle mass
x Demonstration of internal fat (omentum),
bowel loops or abdominal organs (liver)

Mediastinal Masses - Summary: Mediastinal Masses - Summary: Non-
Pathognomonic Surgical Lesions
x Lateral thoracic meningocele x Advanced lung cancer
x Extramedullary hematopoiesis x Metastatic lymphadenopathy
x Aneurysm x Lymphoma
x Paraesophageal varices x Malignant germ cell neoplasm
x Teratoma x

x Lipomatosis x Castleman disease
x Congenital cyst (bronchogenic, x Paraesophageal varices
pericardial) x Morgagni hernia (if asymptomatic)
x Mediastinal goiter
Mediastinal Masses - Summary: Surgical
Mediastinal Masses - Summary: Cystic Lesions
x Thymoma (mural nodules) x /! !
x [!

Q
x /!

Q+
posterior mediastinum - carinal) resectable)

x Pericardial cyst – right cardiophrenic x Mature teratoma
angle, water attenuation content, x Neurogenic neoplasm
imperceptible wall x Congenital cyst (if symptomatic)
x Thymic cyst (multilocular, anterior x Mediastinal goiter (if airway compromise)
mediastinum) x ] !

!
x Neurogenic neoplasm (paravertebral, x Aneurysm (if large, symptomatic or
heterogeneous, osseous erosion) complicated)
¾Meningocele (NF1, continuity with
spinal canal, homogeneous water Approach to Mediastinal Masses
V x Clinical
x Mature teratoma (multilocular cystic ¾~ !
Q
V
lesion with fat content) Symptoms
x Lymphoma (lymphadenopathy with cystic x Radiography
change) ¾Mediastinal compartment
x Lymphangioma (multilocular cysts with ¾Diffuse mediastinal enlargement vs.
extrathoracic extension) focal mass
x Achalasia (enlarged esophagus with ¾

internal debris, imperceptible gastric x Cross-sectional Imaging
bubble) ¾$
!

x Mediastinal goiter (high attenuation, normal structures

continuity with thyroid) ¾! $

Mediastinal Masses - Summary: FAT
x Lipomatosis (diffuse, no mass effect)
x Lipoma
x /!
Q+

+

to thymus)
x Mature teratoma (cystic lesion with fat
content)
x Morgagni hernia (right cardiophrenic
angle, continuous with abdominal fat)
Mediastinal Masses - Summary: Intense

Enhancement
x Mediastinal goiter (continuity with cervical
thyroid)
x Hemangioma (phleboliths, follows
vascular enhancement)
x Castleman disease (enhancing
lymphadenopathy)
x Paraganglioma (hypertension,
catecholamine production)
x

Varices (enhancement of
vascular lumen)

References
General
1. Aquino SL, Duncan G, Taber KH, Sharma A, Hayman LA. Reconciliation of the anatomic, surgical, and
!

+
!
&
\

/
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2.
&

!
&
'

}
~

#
~
&
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+
~
+
!
!&
/!
&
]

JKKK@^Z=Z=J&
3.
[&
!
&
!!
$
<=^XXZK_JK&
4. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior compartment.
'

$
²
]

³
~
&

³
~
+
~
+
!
!
!
&
!!
$
<===JZ^`=X^&
5. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the middle-posterior

&
'

$
²
]

³
~
&

³
~
+
~
+
!
!&
!
&
!!
$
<===@J=XZ^X&
6. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the paravertebral region.
'

$
²
]

³
~
&

³
~
+
~
+
!
!&
!
&
!!
$
<===@J=^_ZX&
7. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics
JKKJ@JJ$^=$=X&
8.

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]&

~
+
!
&
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$

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]

}&
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+
!
' &
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/!
JKK@J<^KJ&
9.
\
$
]#&
#
/
+
/
+
!
/! &
'
#

+
/
$
&
*

$
<===&
10. $!

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+
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!

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/
+
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+
J<

X&
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~

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!

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+
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<==^XXJ_^&
11. Shimosato Y, Mukai K. Tumors of the mediastinum excluding the thymus, heart and great vessels.
'
$!

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/
+
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J<

X&
!
~

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<==^@J_=^X&
12. $
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+
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<===@<_<`J<^<&
13.
/
[
|&
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+
#

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&
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"+
:

<==_X^K<&
Thymoma
14. Rosado-de-Christenson ML, Galobardes J, Moran CA. /!

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&
}!
<==J@<J<`<Z&
15. Thomas CR, Wright CD, Loehrer PJ, Sr. /!

+
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<===@<^JJZKJJZ=&
16. /

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::
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Thymic Malignancy
17. \
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18. !
]

}
q

}:
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/:
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/!

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<===@<=^K^X&
Thymolipoma
19. Rosado-de-Christenson ML, Pugatch RD, Moran CA, Galobardes J. /!

+
J^
&

<==_@<=X<J<&
Thymic Hyperplasia
20. Budavari AI, Whitaker MD, Helmers RA. Thymic hyperplasia presenting as anterior mediastinal mass in 2

*!
}:
&

JKKJ@^^_=`=&
21. Hara M, McAdams HP, Vredenburgh JJ, Herndon JE, Patz EF Jr. Thymic hyperplasia after high-dose
!!

*!

&
\
<===@<^X<X_<_.
Germ Cell Neoplasms
22. !
$\
]
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/#&

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++
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<==Z@<^<`=<_&

23.
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Lymphoma
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26.
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$
Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition.
!!
$
<===@<J=XXK&
Non-Neoplastic Lymphadenopathy
27.

q
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| :
[

$&
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&
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28.
#

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]
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&

+
!
!"

+
*!

!!
&

<==Z@JK=JJ<Z&
29. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis.
}!
JKK<@J<^X^`^&
Mediastinal Cysts
30. !

#
\
$

&
'!

!

/
+
*!

!!
&
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JKK<@<^^ZZ<`&
31. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics
JKKJ@JJ$^=$=X&
32.
#
q

!
]

$&
[!

+
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!!
&

JKKK@J<^__<&
Neurogenic Neoplasms
33. Ichikawa T, Ohtomo K, Araki T, et al. }

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<==@=<<_J<&
34. !:
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+
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:

&
$
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<===@<`
78.
35.

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36.
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Endocrine Lesions
37. [
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5.
38. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior mediastinal
&
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39.

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~
+
~
+
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$
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937.
40. Hopkins CR, Reading CC. /!

!
&
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<==`@<J^==`&
Vascular Lesions
41. !
]

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42.
#
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#
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43.
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44. $!++

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<==_@<JJZX=&
45. Henseler KP, Pozniak MA, Lee FT Jr, Winter TC III. Three-dimensional CT angiography of spontaneous

!&
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JKK<@J<=<^K_&
46. '

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47. '

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48.
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49. Lee SJ, Lee KS, Kim SA, Kim TS, Hwang JH, Lim JH. Computed radiography of the chest in patients with
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Miscellaneous Lesions
50.
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1999;2987-3010.
51. Mueller CF, Klecker RJ, King MA. Case 3. !&
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54. Gilkeson RC, Basile V, Sands MJ, Hsu JT. Chest case of the day. Extramedullary hematopoiesis (EMH). AJR
<==^@<=J^
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55. Moellers M-C, Bader JB, Alexander C, Samnick S, Kirsch C-M. Localization of extramedullary
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Chest Seminar: Where is the Lesion?
Melissa Rosado de Christenson, MD, FACR
Learning Objectives x Carcinoid
x To review the radiographic features of ¾[

!
thoracic radiologic abnormalities based on x Solitary metastasis
location ¾]*

!
x To enumerate the radiologic x #

'+
characteristics that allow lesion ¾Borders
localization and the formulation of a
focused differential diagnosis
Case 1: 38-Year-old Woman With Cough

x Location
x Differential diagnosis
x Next best study
x ~
Solitary Lung Mass

x Young, relatively asymptomatic woman
x
*!
*

x Lower lobe location
x Abutting bronchus
Case 2: 16-Year-old Girl With Cough

x Location
x Next best study
x ~
Solitary Lung Mass

x Lung cancer
¾$q

+
¾Stage ?
Chest Seminar: Where is the Lesion 148 Chest Radiology

Anterior Mediastinal Mass
x Young girl, relatively asymptomatic
x

*!
!

x Central water attenuation
x Heterogeneous mural nodule
Anterior Mediastinal Mass

x Mature teratoma
¾

+

¤¤
x Thymic cyst
¾

¤¤´

x Lymphoma
¾Correct age group … but no
lymphadenopathy
x Thymoma
¾Fluid, calcium, mural nodule … but fat
attenuation
Case 3: 58-Year-old man With Chest

Pain and Hemoptysis
x Location
x Next best study Lung/Chest Wall Mass With Cavitation
x ~ x Bronchogenic carcinoma
¾Chest wall invasion
Lung Mass With Cavitation and Chest ¾Stage?
Wall Involvement x Infection
x Symptomatic older man ¾Actinomycosis, tuberculosis, fungus
x Chest wall involvement (rib destruction) x
!
*

¯
x Upper lobe location rarely produces cavitation

x Cavitation; thick nodular cavity wall x Other
Chest Radiology 149 Chest Seminar: Where is the Lesion

Case 4: Asymptomatic 40-Year-old Man;
Pre-Operative Radiograph
x Location
x Next best study
x ~
Multifocal Pleural Nodules

x Asymptomatic patient
x No known malignancy
x
!

x

abnormalities?
Multifocal Pleural Nodules

x Splenosis
¾Where is the spleen?
x Pleural metastases – may be unilateral
x Malignant pleural mesothelioma –
typically with circumferential nodular
pleural thickening
x Other
Splenosis
x Auto-transplantation of splenic tissue
following splenic rupture
x

+

peritoneal nodules
x /!

¾Splenic rupture with diaphragmatic

disruption
¾Multiple pleural-based nodules
¾May be missed on radiography
¾99mTC-tagged heated RBC
scintigraphy
¾Liver-spleen scan
Case 5: 34-Year-old Man With Left Chest

Pain for Many Years
x Location
x Next best study
x ~
Chest Seminar: Where is the Lesion 150 Chest Radiology

Multifocal Chest Wall and Mediastinal
Masses
x Chronic minimal symptoms
x Is involvement unilateral or bilateral?
x Benign pressure erosion
x Pulmonary involvement
x !
!
*

involvement
Multifocal Chest Wall and Mediastinal

Masses
x
¾Malignant potential
x Vascular lesions
¾Arteriovenous malformations
¾Collateral vessels
x Metastases
x Other
Chest Radiology 151 Chest Seminar: Where is the Lesion

Pneumonia: Usual and Unusual Organisms
Rosita M. Shah, MD
Pulmonary Infections Alveolar Filling Pneumonias
x x

¾Pattern approach ¾Early imaging
x Clinical settings ¾Normal lung structure
¾Community-acquired Pneumonia x Organisms may produce more than one
¾Healthcare related Pneumonia pattern
x Unusual Pulmonary Infections x Basic pattern differentiation may be
+
Pulmonary Infection: C
" ¾Interstitial vs bronchopneumonia
Morphology
x 3 radiographic and pathologic patterns Community-Acquired Pneumonia
¾Lobar x Epidemiology
¾Lobular (bronchopneumonia) ¾J¯<K
<KKK

¾Interstitial ¾22-50% hospitalization rate
Outpatient mortality 1-5%
Pulmonary Infection: C
Inpatient mortality 25%
x Lobar and lobular pneumonias both x Etiology

¾'

`K

:

x $
++
isolated
¾$
+

¾

of spread S. pneumoniae
¾~
+

M. pneumoniae
¾Radiographic pattern K. pneumoniae
¾Etiologic agents ~"Q?
L. pneumophila
Alveolar Filling Pneumonias Respiratory viruses
x Site of initial infection and pattern of
spread S. pneumoniae: Demographics
¾Lobar pneumonia x S. pneumoniae most frequent isolate in
Peripheral alveoli CAP
Intra-alveolar spread ¾Up to 75% incidence
¾Bronchopneumonia x Recognized risk factors
Terminal bronchioles and ¾Alcoholism, splenic dysfunction, viral
centrilobular acini pneumonia, congenital and acquired
Bronchiolar spread

x 25-40% mortality, unchanged >30y
Alveolar Filling Pneumonias
x ~
+

S. pneumoniae: Pathology
radiographic pattern x Aspiration to peripheral air spaces
¾Lobar pneumonia x Alveolus represents site of initial

x Spread occurs by contiguous involvement
¾Bronchopneumonia of adjacent alveoli
'

x 3 pathologic stages
Heterogeneous opacity
S. pneumoniae: Pathology
Alveolar Filling Pneumonias x CONGESTION
x Etiologic agent may vary ¾Increased capillary permeability
¾Protein rich edema
¾
:

:

+
Kohn and Canals of Lambert

x RED HEPATIZATION
¾

:
hemorrhage
x GRAY HEPATIZATION
¾!

+
blood products
Pneumonia 154 Chest Radiology

S. pneumoniae: Radiology [Figure 1] M. pneumoniae: Pathology
x LOBAR pattern x Eaton agent-1944
¾#

¾}

¾Nonsegmental distributions ¾Absent cell wall
x Spread at alveolar level results in x Acute cellular bronchiolitis
nonsegmental distributions characteristic ¾¤
[!
of early lobar pneumonia ¾¤
'

x ‘Round’ pneumonia
¾Manifestation of nonsegmental M. pneumoniae: Radiology [Figure 2]
distribution x LOBULAR pattern Bronchopneumonia
¾Most common in pediatric infection ¾Heterogeneous, patchy consolidation
with S. pneumoniae Minimal exudate into centrilobular
alveoli
¾Segmental
Peribronchial spread
Figure 1 ¾Volume loss
¾Minimal air bronchograms
S. peribronchial thickening
pneumoniae
with lobar
pattern
consolidation.
Figure 2
M.
pneumoniae
with
broncho-
S. pneumoniae: Radiology pneumonia
x LOBAR pattern pattern.
¾Prominent air bronchograms
¾Preserved volume
S. pneumoniae: Radiology
x 48% of consecutive hospitalized pts
demonstrated focal lobar patterns M. pneumoniae: Radiology
x 33%, multifocal lobar patterns [Figures 3 and 4]
x 16% lobular pattern x CT Findings
x Dominant pattern did not vary with ¾86% centrilobular nodules
immune status or disease severity ¾82% bronchovascular thickening
AJR 2000;175:1533. ¾59% consolidation with lobular
distribution
S. pneumoniae: Radiology Reittner AJR 2000;174:37.
x Infrequent cavitation
¾Associated with serotype 3
x Most frequent organism in pulmonary
gangrene
¾Vascular thrombosis from severe
necrosis
¾Intracavitary mass (sloughed lung)
M. pneumoniae: Demographics
x 15-35% of CAP Figure 3
¾50% of CAP during summer months
x Peak age 5-25 yo M.
x Self limited pneumoniae
¾Few fatal cases associated with ARDS with
widespread
¾Increased severity in sickle cell centrilobular
anemia ground-glass
x Atypical Pneumonia Syndrome nodules on
¾Prominent extrapulmonary complaints HRCT.
Chest Radiology 155 Pneumonia

Figure 6
Figure 4 Q?

A interstitial
M. pneumonia,
pneumoniae mimicking
with pulmonary
widespread edema on CXR
centrilobular and reticular
ground-glass interstitial
nodules on abnormality
HRCT. and ground
glass opacity
on HRCT.
Respiratory Viruses
x 'q
[
x q
x Respiratory syncytial virus
RSV 31 Year old hx MDS sp AlloBMT
[Figures 7 to 9]
x Adenovirus
x Herpes viruses
I

$ A: Demographics
x 10-20% CAP Figure 7
x <KKKK_KKKK
!
q
RSV
epidemic bronchiolitis.
x Peak incidence
¾Pediatric population
x Highest mortality-adult and aged
¾Superinfection S. aureus, S.
pneumoniae
I

$ A: Pathology
x Type 1 pneumocyte necrosis Figure 8
x Type 2 pneumocyte proliferation
RSV
x

bronchiolitis.
x Viral inclusions
x Mucosal ulceration predisposes to
bacterial infection
I

$ A: Radiology
[Figures 5 and 6]
x INTERSTITIAL pattern
¾
¾Peribronchial thickening
¾GGO Figure 9
¾Symmetric, parahilar distribution
RSV
bronchiolitis.
Figure 5
Q?
A interstitial
pneumonia,
mimicking
pulmonary
edema on
CXR and
reticular
interstitial
abnormality
and ground
glass opacity
on HRCT.

Adenovirus [Figure 10] Severe Community-Acquired Pneumonia
x Interstitial pneumonia x ~
x Necrotizing bronchiolitis ¾Impending respiratory failure
x Potential infection in immune competent ¾Hemodynamic instability
and suppressed hosts ¾Radiographic assessment
¾Military epidemics Bilateral or multilobar involvemnt
¾Pediatric population 50% increase in size of opacity
¾Transplant recipients within 48 hours
x Swyer James, Macleod’s Syndrome x Etiology
¾Bronchiolitis obliterans following viral ¾S. pneumoniae
infection in early childhood ¾L. pneumophila
¾Lung hypoplasia ¾S. aureus
¾Bronchiectasis 20 to longstanding BO ¾P. aeruginosa in patients with
bronchiectasis, HIV
L. pneumophila: Demographics
x 15% of CAP
Figure 10
x Epidemic and sporadic infection
Swyer-James ¾HAC systems
syndrome x Legionnaire’s disease = pneumonic form
following x Peak summer
childhood
viral
x Prominent extrapulmonary symptoms
pneumonia, ¾CNS, GI or renal manifestations
with x Acute onset, 10% mechanical ventilation
longstanding x 15% mortality in cases requiring
left lung hospitalization
bronchiolitis
obliterans and associated bronchiectasis.
L. pneumophila: Pathology
x [!

x Gram - bacillus
Respiratory Herpes Viruses
x HSV-1, HSV-2, VZV, EBV, CMV L. pneumophila: Radiology [Figure 12]
¾Primary infection, latency, reactivation x Bronchopneumonia pattern
¾Up to 40% mortality ¾Bilateral and multifocal 50%
x Risk factors x Pleural effusions 60%
¾Immune-suppression, lung x May produce lobar or mass-like
transplantation, airway management, consolidation
pregnancy x Cavitation uncommon without
immunosupression
Varicella Pneumonia [Figure 11]
x Delayed resolution
x Complication of adult chickenpox
¾5-50% incidence
x Prominent acinar opacities
¾5-10mm nodules, coalescence
¾Patchy GGO
x
!
*!

Kim AJR 1999;172:113
Figure 12
Figure 11
Legionella
Varicella broncho-
pneumonia pneumonia
with and pleural
prominent effusion.
acinar
nodular
pattern.

K. pneumoniae: Demographics
x Nosocomial or community acquired
infection
x Males, > 60 years old
x
+
!
~
~ Figure 15
x 50% mortality
K.
pneumoniae
K. pneumoniae: Pathology resulting in
x Gram -- bacillus progressive
¾

+
:
necrotic
edema pneumonia
¾Lobar expansion - Friedlander’s with
pulmonary
pneumonia gangrene CT
¾Massive necrosis demonstrates
¾Common association with gangrene $Q+
replacing
K. pneumoniae: Radiology >%"
[Figures 13 to 15]
x 5-10% lobar pneumonias
x Lobar pattern S. aureus - Demographics
¾[
x 30-50% colonization rates in healthy
x Abscess 30-50% adults
x Necrotizing pneumonia at CT ¾DM
¾low density areas with small cavities ¾IVDA
Moon JCAT 1995;19:176. ¾HIV
¾Surgical pts
x Methicillin resistance 1944
¾Increasing incidence of resistant
community-acq infection
x Antecedant viral pneumonia
x Frequent cause of health-care related
infection
x Extremes of age
Figure 13 ¾Nursing home population
x Risk factors
K. pneumoniae
resulting in ¾Debilitated states, mechanical
progressive ventilation, burns, indwelling
necrotic catheters, IVDA
pneumonia.
S. aureus: Radiology
[Figures 16 to 22]
x Aerogenous infection results in
bronchopneumonia pattern
x Hematogenous infection
¾multifocal, discrete nodular or wedge
shaped abnormality with normal
intervening lung
x :
QJ`^`V
x Pneumatoceles (60% ped infection)
Figure 14 ¾Ball-valve obstruction
¾Absent destruction
K. pneumoniae
resulting in
x Pleural complications (50%)
progressive necrotic
pneumonia.

Figure 20
S. aureus
septic
empyema.
Figure 16 Follow
up shows
S. aureus
elarging left
necrotizing
upper lobe
broncho-
consolidation
pneumonia.
due to septic
emboli and
right loculated
effusion
representing
empyema.
Figure 21
Figure 17 S. aureus
associated
S. aureus with
necrotizing pneumatocele
broncho- formation on
pneumonia. follow up.
Figure 22
Figure 18
S. aureus S. aureus
necrotizing associated
broncho- with
pneumonia. pneumatocele
formation on
follow up.
P. aeruginosa in Cystic Fibrosis

x Chronic colonization with P. aeruginosa
¾Mucoid variant
¾ABX resistance
¾Elastase production by P. aeruginosa
associated with bronchiectasis
The Practical Points

x S. pneumoniae and K. pneumoniae most
commonly associated with lobar pattern
Figure 19 and pulmonary gangrene
x M. pneumoniae, L. pneumophilus most
S. aureus commonly associated with broncho-
septic emboli.
Nodular
pneumonia pattern and atypical
and wedge pneumonia syndrome
shaped x Viral pneumonias associated with
peripheral interstitial pattern
consolidation x Pathologic in immune-competent and
representing
septic emboli. suppressed hosts
x Prominent bronchiolitis seen with
mycoplasma, adeno and other respiratory
viruses

Healthcare-Acquired Infections Ventilator-Associated Pneumonia (VAP)
x J&`

#'

x Most common nosocomial infection in ICU
¾Primary or contributing COD 100,000 ¾3-21x greater incidence in intubated
¾¶_&`

patients
x Nosocomial pneumonia is the most ¾> 1 intubation, >3 days
common cause of death from HAI ¾10-65% ICU patients acquire VAP
x Rising incidence parallels usage of ¾<XX_
~$

j
antibiotics x 20-80% mortality rate
x Gram negative infections x

¾50% nosocomial pneumonia ¾Organism
¾75% ICU pneumonia # !

P. aeruginosa,
Jarvis Emerg nfect Dis 2001, C+ MRSA
2005. ¾Population
# !

'
Nosocomial Pneumonia: D

<KJK

'
x Pneumonia developing > 48hr sp ¾Late onset
admission, intubation or discharge MDR
Microbiology Nosocomial Pneumonia and Aspiration

[Figures 23 and 24]
x

x S. aureus x Pneumonitis (Mendelson Syndrome)

x Gram – bacilli ¾#
®
J&`
¥K&X

x Anaerobes x [!

x Legionella ¾1-2 hr permeability edema
x Respiratory viruses ¾_
!

¾Rapid clearing
Microbiology ¾30% mortality ARDS
x Early < 5d x Aspiration accounts for up to 15% of CAP
¾S. pneumoniae
¾~"Q?
¾S. aureus Figure 23
x Late > 5d
Aspiration
¾S. aureus pneumonia
¾P. aeruginosa culture
¾Enterobacteriaceae positive for
¾Acinetobacter spp. S. aureus.
¾Stenotrophomonas maltophilia
Pathophysiology
x Direct inhalation
x Hematogenous spread
x Aspiration
¾45% incidence in sleep Figure 24
¾

"

}'
Aspiration
motility pneumonia
¾}

|/ culture
positive for S.
x Secondary airway colonization aureus (top),
¾25% 24hr complicated
¾40% 7d by ARDS 1
x Gastric alkalinization week later.
x Serious illness
x Antibiotic TX
Johanson Ann ntern Med 1972
Pathophysiology P. aeruginosa
x Repetitive aspiration leads to ¾Most common ICU isolate
¾Bronchiolitis ¾70% TX failure rate
¾Lobular (broncho) pneumonia
Peribronchiolar neutrophilic P. aeruginosa: Pathology

Q<K_
+ V x Micro-abscesses
x Necrotic vasculits of sm-med pulmonary
Nosocomial Pneumonia in the ICU arteries
x Hemorrhage

P. aeruginosa: Radiology x Extensive necrosis
x Bronchopneumonia pattern ¾May mimic M. TB or fungal infection
¾Discrete nodules may be indicative of
vasculitis N. Asteroides: Radiology
x Frequent cavitation [Figure 26]
x
++ x Extrapulmonary disease 50% with 40-
90% mortality
Nodular or Mass-Like Consolidations ¾CNS 25%
x Nonsegmental distribution ¾Skin and subcutaneous abscesses
¾‘Round’ pneumonia x Consolidation
x Granulomatous infection ¾Mass-like
¾M. tuberculosis ¾Cavitary
¾Fungi x Pleural and chest wall involvement 30-
¾Actinomycosis 50%
¾Nocardia
A. Israelii: Demographics
x

x $
+
+
¾Cervicofacial 55%
¾Abdomen 20%
¾Pulmonary 25%
x
+

!

Figure 26
Smego RA. Clin nfec Dis 1998;26:1255.
A. Israelii: Pathology N. asteroides

producing
x Multifocal abscesses cavitary and
x Interconnecting sinus tracts noncavitary
x Sulphur granule mass-like
¾Spoke-wheel arrangement of consolidations.
!

organism
A. Israelii: Radiology [Figure 25]

x Consolidation
¾Mass-like Parasitic Infection
¾Cavitary x Pulmonary involvement due to
x Pleural, chest wall and osseous hypersensitivity or direct invasion
involvement ¾Echinococcosis
¾up to 50% ¾Paragonimiasis
¾Ascariasis
Figure 25 ¾Strongyloidiasis
A israelli x !

:
*!
producing other infections

left upper ¾
!

lobe
consolidation ¾Reticulonodular opacities
with ¾Bronchopneumonia
extension ¾Atelectasis
into the
anterior Echinococcus granulosus [Figure 27]
chest wall.
¾Cestode (tapeworm), endemic to S.
America, Australia, Middle East, Africa
N. Asteroides: Demographics and Mediterranean
x Ubiquitous distribution ¾~:
!

*+
x 50% of patients are immunocompetent ¾Intermediate host - sheep, cow, deer,
x
+ moose
¾Neutropenia x Duodenum - portal venous system - liver
steroids, late HIV, hematologic ¾45-75% isolated liver involvement
malignancy, alveolar proteinosis ¾15-35% pulmonary involvement
x Pulmonary cysts acquired in childhood
N. Asteroides: Pathology x Diagnosis 30-40yo
x Peribronchial abscesses, granulomatous x Intact cyst - asymptomatic
x Eosinophilia 25-40%

Ascariasis Lumbricoides
x Roundworm infection
x Most common parasitic infection
Figure 27
¾Endemic worldwide
E. granulosus ¾25-95% prevalence
with intact ¾Highest incidence in children
(right lung) x Large ingestion associated with
and ruptured pneumonitis
(left lung)
cysts. x Small bowel – systemic circulation –
alveoli – trachea – small bowel
Strongyloides Stercoralis
x Round worm
x Skin – systemic circulation – alveoli –
trachea – small bowel
Echinococcus granulosus: Pathology Ascariasis Strongyloides: Radiology

x Hydatid cyst consists of 3 layers x Bronchopneumonia
¾

!

x Patchy, transient consolidation
¾Exocyst – acellular laminated x Eosinophilic pneumonia
membrane
¾|

B. Anthracis: Anthrax [Figures 28 and 29]
daughter cysts x }¤

+

¾Dormant spores are virulent
Echinococcus granulosus: Radiology x Infection typical in livestock
x Intact cyst x Exotoxin production associated with
¾Well demarcated, homogeneous mass hemorrhagic mediastinitis, edema and
¾Spherical when central, ovoid when pleuritis
peripheral Earls Radiology:222:305, 2001.
¾Multiple 20-30%
¾Lower lobes 60%
x Impending Rupture
¾Crescent sign - air between pericyst
and laminated membrane
x Ruptured cyst
Figure 28
¾Water lily sign - rupture of endocyst
B. anthracis
Paragonimiasis Westermani producing
x /
¯

pumonary
¾Endemic to Asia lymphedema
¾Contaminated freshwater crab (top) and
x Jejunum – peritoneal cavity – diaphragm hemorrhagic
mediastinal
– pleura – lung adenopathy
x Chronic granulomatous reaction (bottom).
Paragonimiasis Westermani: Radiology

x

+
infection
¾PTX and pleural infection during
pleural penetration by juvenile worms
¾Transient patchy consolidation and Figure 29
linear tracts during larval migration
B. anthracis
¾Peribronchial cysts associated with producing
mature worm pumonary
lymphedema
(top) and
hemorrhagic
mediastinal
adenopathy
(bottom).

The Role of Imaging in Pneumonia The Practical Points
x Diagnosis of infection x Organisms may produce more than one
¾Presence of centrilobular nodules in pattern
acute parenchymal disease favors ¾Bacterial, viral and fungal pneumonia
pneumonia !:

/

¾Thin section CT allows earlier diagnosis transplantation

of pneumonia in immunosuppressed x Consider clinical setting
pts (5 days) Collins AJR 2000;175:811.
x Recognition of complications
¾Decreased enhancement in pneumonia
indicates severe necrosis
Donnelly LF. Radiology 1997;205:817.

Heussel CP. AJR 1997;169:1347.
Tomiyama N. AJR 2000;174:1745.
References
1. #
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2. Muller NL, Franquet T, Lee KS. Imaging of Pulmonary Infections Lippincott Williams & Wilkins 2006.
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Uncommon Malignant Tumors of the Lung
Gerald F. Abbott, MD
Uncommon Malignant Tumors of the Neuroendocrine Cells and Tumors:
Lung: Electron Microscopy
x Bronchial Carcinoid - most common x Cytoplasmic neurosecretory granules
“Uncommon” x Central or eccentric dense core
x Adenoid Cystic Carcinoma ĺSalivary x Thin lucent halo
Gland Tumors x May contain biologically active peptides
x Mucoepidermoid Carcinoma ĺSalivary
Gland Tumors Neuroendocrine Markers:
x Carcinosarcoma ĺMixed Tumors Immunohistochemistry
x Pulmonary Blastoma ĺMixed Tumors x Chromogranin
x Synaptophysin
“Bronchial Adenoma”: History Lesson x Neural cell adhesion molecules (NCAM)
x /
+
+

¾Bronchial Carcinoid Carcinoid: Relationship to Small Cell
¾Adenoid Cystic Carcinoma Carcinoma
¾Mucoepidermoid Carcinoma x Similarities
x A misnomer ¾Neurosecretory granules
x These tumors are not benign ¾Rosette and trabecula formation
x Differences
Carcinoids ¾Fewer granules in Small Cell
x Gastrointestinal tract 90% Carcinoma
x Lung ¾Carcinoid not associated with smoking
x Thymus
x Biliary tract Tumors with NE Morphology
x Ovarian teratomas x A spectrum by light micropscopy
Bronchial Carcinoid x Low-grade Typical carcinoid

x Typical carcinoid x Intermediate Atypical carcinoid
¾Low-grade malignancy x High-grade Small cell lung ca
x Atypical carcinoid x Large cell neuroendocrine
¾Moderate-grade malignancy carcinoma
Typical Carcinoid Neuroendocrine Tumors: World Health

x 0.6-2.4% of all pulmonary neoplasms Organization Criteria (1999)
x Low grade malignancy x Typical carcinoid <2 mitoses per 10 HPF
x Good prognosis x Atypical carcinoid 2-10 mitoses per 10
¾=`
:
:: HPF
x Not associated with smoking x ]

<<
or more mitoses per 10 HPF (median 70)

Typical Carcinoid: Demographics x $

<<

<K
x Males = Females HPF (median 80)

x

&

`K

x $
!
!

Typical Carcinoid: Microscopy

x Uniform cells
x Forming nests, ribbons, rosettes,
trabeculae
x Stroma highly vascular
x
"!

metaplasia
x Polygonal cells, pale cytoplasm, stippled
nuclear chromatin
x Rare mitoses
x

>

166 Chest Radiology
Atypical Carcinoid: Histopathologic Bronchial Carcinoid: Central
Criteria x Bronchiectasis
x Poor architectural organization x Mucoid impaction
x Cellular pleomorphism x Obstructive pneumonia
x Focal necrosis
x Increased mitotic activity Bronchial Carcinoida: Peripheral
Arrigoni J. Thorac Cardiovasc Surg 1972.
x Late presentation
x Discovered incidentally
Atypical Carcinoid
x Morphology between Typical carcinoid and
Bronchial Carcinoid: Radiographic
Small Cell Ca
Findings [Figures 2 and 3]
x Tend to be larger, more invasive,
x Central tumors in 80%
peripheral
x Lobar, segmental, subsegmental bronchi
x
~

!
/
x Consolidation, atelectasis
x $

/
x Pleural effusion
x '

/
Atypical Carcinoid
x 10% of bronchial carcinoid
x Peripheral
x Increased mitoses
x Aggressive behavior. Early mets Figure 2
x Osteoblastic bone metastases
x !
~~"
$

Bronchial
carcinoid
manifesting as
Bronchial Carcinoid: Gross Pathology a central, well-
[Figure 1]
x Usually seen at bronchoscopy perihilar mass.
x $+
!
!

x Sessile. May be pedunculated
x Often extend through wall
Figure 3
Small endobronchial carcinoid tumor in distal left

mainstem bronchus.
Figure 1
Growth patterns of bronchial carcinoid tumors. Partially

`

{

common.
Chest Radiology 167 >

Bronchial Carcinoid: CT Features
[Figures 4 to 6]
x Bronchial relationship in 83%
x Partially endobronchial
x Completely endobronchial
x Abutting a bronchus
x Sharply marginated, lobulated mass
x
!

¤¤
x Atelectasis, consolidation, bronchiectasis,
mucoid impaction
x Lymphadenopathy
Figure 6
Bronchial carcinoid in a 34-year-old male.

> `{

distal, peripheral consolidation. Contrast enhanced CT
(right) shows diffuse contrast enhancement.
Bronchial Gland Tumors

x Adenoid Cystic Carcinoma
x Mucoepidermoid Carcinoma
x Equivalent to salivary gland tumors of
Figure 4 same name
Bronchial Carcinoid. Adenoid Cystic Carcinoma [Figure 7]

Partially endobronchial (‘iceberg’) tumor in left upper x Synonym - Cylindroma
lobe. x 80% of bronchial gland tumors
x 20-35% of all tracheal tumors
x Second most common tracheal
malignancy
x (after Squamous Cell Carcinoma)
Figure 7
Figure 5
Adenoid cystic carcinoma partially occluding the

Bronchial carcinoid obstructing left upper lobe trachea and extending into the adjacent soft tissues of
bronchus with distal atelectasis, pneumonitis and the mediastinum.
associated left hilar lymphadenopathy.
>

168 Chest Radiology
Adenoid Cystic Carcinoma
x Guarded prognosis
x Common local recurrence
x Occasional metastases to regional nodes
x Rarely extrathoracic spread
Adenoid Cystic Carcinoma:

Demographics
x Males = Females
x Wide age range. Average 40 - 50 years
x $

!
*!q
dyspnea, hemoptysis
Adenoid Cystic Carcinoma: Microscopy

x Mucin-containing cysts
x Varying in caliber
x Within larger tumor tubules
x Surrounds, invades nerves
x |
:

!
x Few mitoses
Adenoid Cystic Carcinoma: Pathologists’

Pitfalls
x Misdiagnosis
¾Adenocaracinoma
¾Pleomorphic adenoma
¾Small cell carcinoma
x Metastatic salivary gland tumor Figure 8
x Solid pattern on small biopsy Adenoid Cystic Carcinoma.
Coronal (left) and saggital (right) surface-rendered
Adenoid Cystic Carcinoma: Gross CT images of the trachea demonstrate both nodular
x Endobronchial mass and circumferential deformity of the air-column in the
¾Trachea and main bronchi lower trachea.
x Sessile, polypoid, annular growth
x Proximal & distal spread Mucoepidermoid Carcinoma:
x Extension into mediastinum Demographics and Prognosis
x Male = Female. Wide age range
Adenoid Cystic Carcinoma: Radiology x Cough, fever, hemoptysis, pneumonia,
[Figure 8] atelectasis
x
!

! x ]*
"

x Intraluminal nodule or mass x # !

!
x
+
!

!
Bronchogenic Ca
x 10 - 15% in lung periphery
x /

'
!
+
::
Mucoepidermoid Carcinoma [Figure 9]
mediastinal involvement
Figure 9
Mucoepidermoid carcinoma arising in the right

mainstem bronchus at the origin of the right upper
lobe bronchus.

Mucoepidermoid Carcinoma: Microscopy
x ]*
¾Mucinous cysts
¾Solid collections of squamous cells
x # !
¾Solid sheets of tumor

¾Mitoses and necrosis
Mucoepidermoid Carcinoma: Gross

x Submucosal, smooth-surfaced
x Endobronchial, exophytic, polypoid
x High-grade may have ragged invasive
appearance
Mucoepidermoid Carcinoma: Radiologic

Findings [Figures 10 and 11]
x Solitary nodule or mass
x Most in main or lobar bronchi
x Few in trachea
x ~
++
¾Atelectasis, pneumonia
x Central lesions
x Atelectasis, pneumonia
Figure 11
Central mucoepidermoid carcinoma manifesting as

mild prominence of the suprerior aspect of the right
hilum with associated atelectasis of the right upper
lobe.
Mixed Tumors: Neoplasms with

Malignant Epithelial and Mesenchymal
Components
x Carcinosarcoma
x Pulmonary Blastoma
¾Carcinosarcoma
x Rare - 0.3% of all lung neoplasms
x Middle-aged and elderly males
x Poor prognosis
x :

:
*
metastases, rapid death
Carcinosarcoma: Microscopy
x Epithelial component
¾Squamous Cell Carcinoma
¾Adenocarcinoma
¾Undifferentiated Carcinoma
Figure 10 x Mesenchymal component
¾Usually dominant
Mucoepidermoid carcinoma manifesting as a solitary
pulmonary nodule on chest radiography. ¾Spindle cell (common)
¾Chondrosarcoma
¾Osteosarcoma
¾Rhabdomyosarcoma
>

170 Chest Radiology
Carcinosarcoma: Gross Pulmonary Blastoma: Gross
x Peripheral x Large mass
¾Large mass x Unencapsulated and soft
¾Average diameter 6 cms x Abundant central necrosis and
¾Frequent necrosis and hemorrhage hemorrhage
x Central
¾Endobronchial growth Pulmonary Blastoma: Radiology
¾May extend to adjacent parenchyma [Figure 13]
¾Tumor-distended bronchi may x Large peripheral mass
resemble mucus plugs x Well-circumscribed
x May show pleural invasion
Carcinosarcoma: Imaging [Figure 12] x May metastasize
x Peripheral
¾Large
¾Well-circumscribed mass
x Central
¾Atelectasis, pneumonia,
¾Tumor “mucus plugs” Figure 13
¾Upper lobe predominance
¾Direct extension to pleura, chest wall, Pulmonary
blastoma
and mediastinum manifesting
as a large,
heterogeneous
mass in the left
Figure 12
lower lobe.
Carcinosarcoma.
Contrast
enhanced CT
demonstrates a
peripheral mass
with irregular
and lobulated
borders in the Endobronchial Tumors: Malignant
right upper lobe. x Squamous cell ca
x Adenocarcinoma
x Small cell ca (rare)
x Carcinoid
x Adenoid cystic ca
x Mucoepidermoid ca
Pulmonary Blastoma x Carcinosarcoma
x Primary lung tumor x Pulmonary blastoma
x Mix of epithelial and mesenchymal x Sarcoma (10%)
components x Endobronchial metastasis
x Both components blastomatous and x Lymphoid malignancies (NHL > HD)
immature
x Morphologic mimic of embryonal lung
x ? a variant of carcinosarcoma
Pulmonary Blastoma: Demographics

and Prognosis
x Predominantly males
x [!

seventh decades
x $
!
!

chest pain
x Poor survival
Pulmonary Blastoma: Microscopy

x "
¾Epithelial-lined tubules
¾Primitive stroma
x Resembles embryonal lung
x
!
!

mixed

Benign Tumors of the Lung and Tumor-like Lesions
Benign Tumors and Tumor-like Lesions Hamartoma: Microscopic
x Hamartoma x Cartilage nests (lobules) in 95%
x

x $

x '
x Mature fat cells
x Granuloma x Cleft-like invaginations of entrapped
respiratory epithelium
“Hamartoma”
x Albrecht, 1904 Hamartoma: Gross
x Tumor-like malformation x Solitary
x Tissues normal to location x 1 – 3 cm (rarely “Giant”)
x In excess or disarray (disorganized) x Rounded, well-circumscribed, lobulated
x “Adult”, “Classic”, and “Local” hamartoma x Firm lesions. Usually cartilaginous
x May see areas of fat
Hamartoma x Easily “shelled-out”
x Acquired lesion
x Disorganized growth of tissues normally Hamartoma: Radiographic
found in lung x $!

x Benign neoplastic proliferation x Most < 3 cm
x Probably derived from bronchial x

¢
<K<`
wall mesenchymal cell (“benign x Rarely see fat on CXR
mesenchymoma”) x May enlarge on serial CXRs
x Up to 3 to 5 mm per year
Hamartoma
x Most common benign tumor of lung Hamartoma: C
x 77% of benign lung tumors x 10 – 15% - speckled or “popcorn”

x 8% of SPNs x “Popcorn” less frequent than once thought
x 3% of all lung tumors x Diagnostic when present
x Nodular growths within lesion
Hamartoma: Evidence of Acquired x Protrude in different directions
Lesion
x Onset in adult life Hamartoma: Computed Tomograpahy
x Often adults with previously normal CXR x Distinguishes fat and cartilage
x Almost never seen in infants x Most are 2.5 cm or less
x #
:

+
x Smooth edge
epithelium x
+

+

*!

+
x
!
<J

q13-q15 regions (as in other benign soft- x :

tissue neoplasms)
Hamartoma: Computed Tomography
Hamartoma: Demographics [Figures 1 to 3]
x

XK^K
x Thin sections (2mm)
x

!
x Smoothly contoured nodule
x

XJ
Q<<
+
x = or < 2.5 cm diameter focal fat in 8
endobronchial hamartoma) :"

+
*!

x Asymptomatic in 90%
x < 8% obstructive symptoms
Hamartoma: Clinical
x Most are peripheral and asymptomatic Figure 1
x '+

!
x '+
!

Hamartoma.
x Fever, cough, expectoration, chest pain
>
chest CT
demonstrates
a peripheral
solitary nodule
with focal fat
attenuation.
Benign Tumors of the Lung 174 Chest Radiology

Chondroma
x Rare
x Benign cartilaginous tissue
x Parenchymal or endobronchial
x Lack epithelial-lined clefts seen in
hamartomas
x In young female
x Search for Carney’s Triad
Hamartoma: Endobronchial
x Morphologically identical to parenchymal
x Often polypoid. Sessile or thin pedicle
x Manifest by airway obstruction
x

+

+

scant or absent
Hamartoma: Treatment and Prognosis

x Benign
Figure 2 x Surgical excision = Cure
x |"

!
Hamartoma.
>
Papillomas
in the left lower lobe with a lobular area of “popcorn”
x Branching or coarsely lobulated tumor

"
x Arise from and project above an epithelial
surface
x Rare pulmonary tumors
x Solitary (rare) or Multiple (papillomatosis)
x Proximal or peripheral
Solitary Papillomas
x Rare
x Usually in adults
x Papillary exophytic growth
x Trachea, main or lobar bronchi
x Males >40 years of age
x Post-obstructive pneumonia,
bronchiectasis
Juvenile Laryngeal Papillomatosis

x Children 18-months to 3-years of age
x Majority remain localized, disappear
Figure 3 spontaneously
x May spread distally and obstruct airways
Hamartoma. ¾5% Spread remains limited to trachea
>
@ ¾1% Develop lung disease - 10 years

after laryngeal disease (extension
associated loss of volume in the left lung. to bronchi, bronchioles, alveolar
airspaces)
Hamartoma: Computed Tomography
x X

+

Laryngeal Papillomatosis:
x _

++
Demographics and Etiology
x 38% areas of fat x Human papilloma virus - HPV types 6 and
x 21% calcium and fat 11
x
+

+ x 0.1% of infants develop LP. Predilection
+

+
“Carney’s Triad” x 50% of mothers have genital tract
x Gastric smooth muscle tumors involvement
x Extra-adrenal paragangliomas x HPV spread transvaginally at birth
x Pulmonary chondromas x Infects oropharyngeal secretions of child
x Association unclear
x Young females < 20 years Papillomas: Microscopic
x
!:

JX
+
!
x Non-keratinizing squamous cells
x Fibrovascular core
Carney JA. Cancer 1979.
x Form papillomatous projections
Chest Radiology 175 Benign Tumors of the Lung

Papillomatosis: Gross
x *
"
x Protrude into bronchial lumens
x May extend into parcenchyma as nodules
or cavities
Laryngeal Papillomatosis
x Majority remain localized
x 5% spread to trachea and distal airways
Tracheobronchial Papillomatosis
x Many remain limited to trachea
x 1% Develop lung disease
x Patients with lung disease may develop
x Squamous cell carcinoma
Tracheobronchial Papillomatosis:
Pathogenesis of Lower Respiratory
Tract Involvement
x Implantation of inhaled fragments from
larynx ?
x Multifocal viral infection ?
x Trauma-induced by tracheostomy ?
x In children, papillomas in bronchi and Figure 5
lung associated with multiple papillomas
of trachea or larynx Papillomatosis and Squamous Cell Carcinoma.
Contrast-enhanced Chest CT demonstrates central
Papillomatosis: Imaging squamous cell carcinoma in the left lower lobe with
distal pneumonitis.
[Figures 4 and 5]
x
*

x Perihilar, Posterior thorax Papillomatosis: Squamous Cell ca
x Grow to several centimeters x Risk for Squamous cell ca 15-years after
x Cavitate, 2 - 3 mm thick walls diagnosis
x
:

: x Risk factors
x :

¾Radiation
¾Papillomatosis ¾smoking
¾Squamous cell carcinoma ¾other carcinogens
¾Abscess (obstructive pneumonitis)
Papillomatosis: Treatment and
Prognosis
x Multiple recurrences
x Multiple excisions
x Tracheostomy
x 37.5% mortality if spread to lungs
x Worse if malignant degeneration occurs
I
Pseudotumor: Synonyms
x Plasma cell granuloma
x Histiocytoma
x Fibroxanthoma, Xanthoma
x

x Mast cell granuloma
I
Pseudotumor
x Uncommon. Reactive or Neoplastic
process?
x May begin as organizing pneumonia
x
!:
:
+
Figure 4 ¾Vascular invasion
¾Vertebral destruction
Papillomatosis. ¾Recurrence
Chest CT demonstrates nodular and cystic opacities
that predominantly involve the dorsal aspects of both
lungs.

I
Pseudotumor I
Pseudotumor: CT
x #
<===
!

[Figure 6]
+

x Solitary nodule or mass
proliferations x Sharply circumscribed
x !
:

+

x Lobulated
cells x Heterogeneous or homogeneous
x Usually solitary, tumefactive lesion x |!

j
x Destroys underlying lung architecture

x Reactive or Neoplastic? x
:
x Endobronchial lesions occur
I
Pseudotumor:
Demographics
x Males = Females
x

<

^^

:
29.5 years
x 60% <40 years
¾!

^

¾Most common 1° lung mass in children

x 74% asymptomatic
x Many patients have history of respiratory
infection
I
Pseudotumor:
Microscopic
x Variable
x A continuum from plasma cell granuloma

!
x "
+

!

cells Figure 6
Q
/

"
I
Pseudotumor: Gross Contrast-enhanced chest CT demonstrates an
x SPN or Mass irregular, heterogeneous mass in the left upper lobe.
x &
&
]
x Lack a capsule
x +
!
!
x <

<K

_&_

I
Pseudotumor: Therapy
I
Pseudotumor and Prognosis
x Solitary nodule or mass in 70% x Diagnosed and treated by surgical
x excision
x May manifest as consolidation x Excellent prognosis after resection
x May mimic neoplasm x Recurrence in 5%
x Endobronchial lesions occur in 10% ¾Especially if mediastinal or chest wall
involvement
I
Pseudotumor: x ~~"

!

Radiographic carcinoma
x $
*

^K
x Endobronchial lesions occur 10% Granulomas
x May extend into mediastinum 5% x Infectious
x Parenchymal consolidation 6% x Sarcoid (necrotizing granulomatosis)
x
:
+ x Hypersensitivity pneumonitis
x May mimic malignant neoplasm
Infectious Granulomas
x Usually no or slow growth. May regress
x Mycobacterial 64%
x Fungal 30%
x Parasitic 6%
Granuloma: Infectious
x Tuberculoma or
x Histoplasmoma
x Satellite lesions common
x Usually small, smooth
x +

+
!
Chest Radiology 177 Benign Tumors of the Lung

Granuloma - Well-D
% Pulmonary
Nodule
x
'

x TB
x Histoplasmosis
x Coccidiodomycosis
x Cryptococcosis
x Aspergillosis
Granuloma - Tiny Nodules

x < 5 mm, micronodular, miliary
x Histoplasmosis
x Blastomycosis
x Coccidiodomycosis
x Cryptococcosis
Solitary Pulmonary Nodule (N = 955)

x Malignant 49%
¾Primary carcinoma 38%
¾Metastases 9%
¾Other 1° malignancy 2%
x Benign 51%
¾Non-neoplastic lesion 37%
¾Tumor
14% ( Hamartoma 8% )
Toomes H. The Coin Lesion of the Lung. Cancer
1983.

Pleural Disease I
Pleural Disease I and II Objectives Pleural Anatomy [Figure 2]
x Anatomy and physiology x Caudal limit of pleura lower than lung
x Non-neoplastic and neoplastic pleural x Costal and diaphragmatic pleura contact
disease to form costophrenic recess
x Chest wall disease
x Radiologic-Pathologic correlation
Pleural Disease I
x Normal anatomy
¾$

¾

x Non-neoplastic pleural disease
¾Effusions
¾Fibrosis
¾Pneumothoraces
Pleural Anatomy
x Parietal Pleura
¾Covers non-pulmonary surfaces
¾$

¾Lymphatics communicate with
¾Pleural space
¾

¾`<`

+

x Visceral Pleura [Figure 1]
¾Covers lung surface
¾~
Figure 2
¾j
: !

¾Lymphatics do not communicate with Pneumothorax in a supine patient manifesting as a

pleural space “deep sulcus” and hyperlucency overlying the left
hemidiaphragm.
Pleural Anatomy
x Junction Lines
x Apposition of layers of pleura
¾Anterior
¾Posterior
Figure 1
Pleural Anatomy - Fissures
x Visceral pleura
Standard (solid lines) and accessory (dashed lines) x Variable depth into parenchyma
" x Complete
x Incomplete
Incomplete Fissures: CT
Pleural Imaging x More frequent on Right
x !

/ x ]

]]

^K
¾Inconspicuous x ]

]]

_^
¾j
¤

K&J
x ]]

]]]

_K
x Thin-collimation x ]

]]]

_
¾1-2 mm thick line
¾Intercostal regions
¾

¾Endothoracic fascia
¾Innermost intercostal m.
# 180 Chest Radiology

Major Fissure: Radiography Accessory Fissures: Radiography
x
QV
[Figure 4]
x Best seen on lateral CXR x <K
¢

JK
/
Q`K
+

x
/_
]+
/`
! specimens)
x !

¾Azygos, Superior, Inferior, Left minor
x Left upper lobe collapse
Major Fissure: CT
x 80-90% on standard CT
¾Lucent band
¾Line
¾Dense band
Figure 4
Major Fissure: CT Propeller-Like
Morphology [Figure 3] Diagram
x
!" illustrates
¾Anterior concave position of

`{&
¾Lateral-facing superior (S)
x '+
!" and azygos
¾Anterior convex (az) accessory
¾Medial-facing "
Accessory Fissures: Azygos

x Abnormal migration of posterior cardinal
vein
x Four layers of pleura
x 1% population
x J

<
Accessory Fissures: CT Azygos

[Figure 5]
Figure 3
Chest CT (lung window) demonstrates right upper

lobe loss-of-volume manifested by displacement of

"*

obstructs the origin of the right upper lobe bronchus.
Standard Fissures: Radiography

x

¾“Lights up” in CHF
¾Interstitial edema
(Subpleural interstitium)
Minor Fissure: CT
x Lucent area
x Devoid of vasculature Figure 5
x 44% triangular
x Z

:
?/

x Ground glass attenuation lateral margin of an azygos lobe.
Incomplete Minor Fissure: HRCT

x Curvilinear line or band
x Increased attenuation
x C-shaped
x
*
]

]
QK=KV
Chest Radiology 181 #

Accessory Fissures: Inferior Accessory Indirect Signs of Atelectasis:
[Figures 6 and 7] Juxtaphrenic Peak
x Separates medial basal segment from x Common in RUL or LUL atelectasis
remaining basilar segments ¾Less common in RML
x Most common x Seen post upper lobectomy
x 30 - 45% anatomic specimens x Small triangular opacity
x ¢
`

<K
x Projects upward from diaphragm
x 80% right-sided x

+

x /
<`
Accessory Fissures: Superior Accessory
x Separates superior segment from basilar
segments
x 6% anatomic specimens
x Right > Left
x Horizontal course
¾'+

Figure 6 Accessory Fissures: Left Minor Fissure
Coned-down
x Separates lingula from remainder of upper
view of right lung lobe
demonstrates an x 8-18% anatomic specimens
inferior accessory x 1.5% of chest radiographs
x Oblique course
the medial basal
segment from the x More cephalad
remaining basal
segments of the Pulmonary Ligament
right lower lobe. x Formed by Parietal & Visceral pleura
x Courses inferiorly & posteriorly
x Contains
x Bronchial veins
x Lymphatics
x Nodes
Pulmonary Ligament: Imaging

[Figure 8]
x ¢

:q
x /
K^K
Figure 8

/
(arrowhead) and right and left pulmonary ligaments
(arrows).
Figure 7

/
(arrow).

Pleural Effusion: Cardiac Pleural Effusion: Empyema [Figure 11]
Decompensation [Figure 9] x Lenticular shape
x Most common transudate x Obtuse margins
x Ç Hydrostatic pressure x Compress lung
x Bilateral >80% x Split pleura sign
x Unilateral = right-sided
x Pseudotumor
Figure 11
Figure 9 Contrast
enhanced chest
Lateral chest CT demonstrates
radiograph smoothly
demonstrates thickened parietal
lenticular and visceral

/
Q pleura enclosing
accumulation in Q

of empyema
(pseudotumor). (“split pleura
sign”).
Pleural Effusion: Bacterial Pneumonia Pleural Effusion: Lung Abscess or

x Parapneumonic effusions 40% Empyema?
x Exudate
x 10% require drainage Pleural Effusion: Empyema
x Complications x Lenticular shape
¾Loculation x Obtuse margins
¾Empyema x Compress lung
x Split pleura sign
Pleural Effusion: Empyema [Figure 10] x ~

!
+

:
x Three phases
¾Exudative Pleural Effusion: Lung Abscess
¾Fibrinopurulent x Round shape
¾Organizing x Does not compress lung
x |
!
+

:
Pleural Effusion: Empyema

x Treatment
¾Tube thoracostomy
¾Fibrinolytics
¾Decortication
Pleural Effusion: Empyema Necessitatis

x Inadequate treatment
x Drainage into chest wall
x Tuberculosis 73%
x [

x Malignancy
x Immunocompromised patients
Figure 10 Pleural Effusion: Tuberculosis

x Exudate
>

Q

/ " ¾ÇLymphocyte count
¾ÈGlucose level
x Unilateral
x Small to moderate

Pleural Effusion: Pulmonary Round Atelectasis: CXR
Thromboembolic Disease x Peripheral mass
x Small x Abuts thickened pleura
x Unilateral x 3.5 to 7 cm
x 30-50% patients x Posterior lower lobe most common
x Exudate 75% ¾Other lobe, diaphragms
x [
++
+ x Bronchovascular bundles converge,
forming “comet tail”
Pleural Effusion: Subpulmonic
x Fluid accumulates between lung base and Round Atelectasis: CT
diaphragm x Rounded subpleural mass
x Shifts apex of diaphragm laterally x Broadly abuts contiguous pleural
x Usually transudate thickening
¾Cardiac decompensation x Air-bronchogram hilar aspect
¾Renal failure x Bronchovascular “comet tail”
¾Cirrhosis with ascites x Loss of volume in same lobe
Pleural Effusion: Subpulmonic Round Atelectasis: Required CT Findings

x Imaging [Figure 12]
¾Apparent elevation diaphragm x Subpleural mass
¾'
!
x Thickened pleura
¾Diaphragmatic spur x Loss of volume
¾
x Comet tail
¾Displace gastric bubble
¾“Rock of Gibraltar” on lateral
Pleural Effusion and Ascites

x CT Features
¾Effusion = outside of hemidiaphragm
¾Ascites = inside of hemidiaphragm
Pleural Effusion: Connective Tissue

Disease
x Rheumatoid arthritis
x Most common thoracic manifestation
x Antedates clinical disease
x |"

! +

*

x Imaging
¾Unilateral
¾Chronic
¾/

¾!"

Pleural Effusion: Asbestos Exposure Figure 12

x Diagnosis of exclusion
x Occupational exposure Chest CT (mediastinal and lung windows)
x No malignancy within 3 yrs

"
x 10 yrs post-exposure
x Exudate
x <X

!:
!

x Recurrent 15 - 30%
x Small (<500 ml) Round Atelectasis: Pathogenesis
x Asbestos exposure
Pleural Effusion - Asbestos Exposure x Pleural effusion
x Associated with diffuse pleural thickening x Atelectasis
¾Involves C-P angle x Pleural adherence
x Implicated in formation of Rounded x Effusion subsides
Atelectasis x Lung re-expands

Pleural Fibrosis Pleural Plaques: Imaging [Figure 14]
x Contraction x Bilateral (80%)
x Pleural Fibrosis x Lateral chest wall
x 2nd most common pleural abnormality x 4th to 8th ribs
x Result of many primary diseases of the x Tendinous diaphragm
pleura x Spares apices and CPAs
x
+

x En face “holly leaf”
x Most localized to single area
x Less often diffuse
¾May have functional abnormalities
Pleural Fibrosis: Focal Figure 14

x Healed Pleuritis
x [

Chest radiograph
x Imaging demonstrates
¾Blunt posteriolateral CP sulci multiple bilateral
¾Rule-out small effusion
plaques.
Pleural Fibrosis: Focal [Figure 13]
x Pleural Plaques
x Serpentine (chrysotile) asbestos
x Dense hyalinized collagen
x Parietal pleural surface
x Asbestos exposure
x Asymptomatic
Pleural Fibrosis: Diffuse Fibrothorax
[Figure 15]
x Fibrous obliteration of normal pleural
space
¾/

¾Hemothorax
¾Asbestos-related pleural effusions
¾Rheumatoid effusions
x j

:

Figure 15
Pleural

"
radiograph
demonstrates
pleural
thickening and

Figure 13 in the right
hemithorax.
Chest CT (lung window) demonstrates multiple
bilateral pleural plaques.

Pleural Fibrosis: Focal x !

x Pleural Plaques ¾Smooth, uninterrupted
x 50% of exposed individuals ¾25% or more of chest wall
x Visible plaques ¾May obliterate c-p suclus
¾<`

¾< -2.0 cm thickness
¾JK

¾¤


x Imaging CT
¾Extends > 8.0 cm cranio-caudal
¾Pleura > 3 mm thick
¾Extrapleural fat hypertrophy
¾¤

¾Mediastinal pleura spared

Pneumothorax
x Air within the pleural space
x Spontaneous
¾Primary
¾Secondary
x Traumatic
Pneumothorax: Primary Spontaneous

[Figure 16]
x

`

<
x 3rd - 4th decade
x Right-side predominance
x 30% ipsilateral recurrence
x 10% contralateral recurrence
x
+

Figure 16
Chest CT
demonstrates left
pneumothorax
and a bleb along
the visceral
pleural surface
of the collapsed
lung.
Pneumothorax: Secondary Spontaneous

x COPD
x Most common concurrent condition
x 0.5% per year
x 45-65 years of age
x Peripheral emphysematous lung
x Mortality rate ~3%

x Pneumocystis Jiroveci Pneumonia (PCP)
x Destruction of alveolar septa ĺ bulla
x Subpleural necrosis ĺcystic degeneration


x Pneumocystis Jiroveci Pneumonia (PCP)
x Complicates in 12%
x Refractory “air-leaks”
x Poor prognosis
x Death in 8 weeks (<57%)

x Lymphangioleiomyomatosis (LAM)
x Women child-bearing age
x Proliferation of smooth muscle
x Bronchiolar obstruction
¾Cysts ĺ PTX
x Recurrence ~40%

x Langerhans Cell Histiocytosis
x Smokers
x Cysts rupture
x Recurrent ptx (25%)

Pleural Disease II and Chest Wall
Malignant Pleural Effusion Localized Fibrous Tumor
x Most common manifestation of metastatic x Rare (< 5% of pleural neoplasms)
involvement x Not related to asbestos
x Exudative effusion x M=F
x Lung Ca 36% x

`K

x Breast Ca 25% x Symptoms in 54%
x Lymphoma 10% x Cough, chest pain, dyspnea
x Ovarian 5% x HPO 0 - 35%
x Gastric Ca 2% x Hypoglycemia 4%
TNM Staging of Lung Cancer Localized Fibrous Tumor: Microscopic

x Malignant Effusion = T4 x Variable patterns
x Disorderly arrangement
x Spindle cells and collagen
x High mitotic activity
x Suggests malignancy 20%
Localized Fibrous Tumor

x Gross
x 2 - 40 cm
x ZK
:

JK

x Lobular, encapsulated
x

x +
*!

hemorrhage, necrosis, cysts
Localized Fibrous Tumor: CXR

[Figures 1 to 3]
x '

Malignant Pleural Effusion:
x Solitary rounded, lobular mass
Diagnosis and Prognosis x Mid to inferior thorax
x Combined pleural cytology and pleural bx
x Obtuse or acute angles at interfaces
x
!

x Pedunculated tumors
x Poor prognosis
¾Positional mobility
x Lung Ca - mean survival 2 to 3 months
¾Pathognomonic
x Breast Ca - mean survival 7 to 15 months
Pleural / Chest Wall Mass

x Discrepant margins on orthogonal views
Figure 1
x Elliptical shapes
x Obtuse angles
Localized
x Cross boundaries Fibrous Tumor.
PA chest
Pleural Neoplasms radiograph
x Primary demonstrates
x ]q

a pleural mass
a peripheral
x Malignant mesothelioma
mass in the
x Secondary right lower
x Pleural metastases hemithorax
¾Bronchogenic carcinoma with incomplete
¾Other carcinomas borders.
¾Lymphoma
¾Invasive thymoma

Localized Fibrous Tumor: Therapy and
Prognosis
x /
+
!

excision
Figure 2
x 90% cure rate
Localized x Symptoms usually resolve post-op
Fibrous Tumor. x Recur with tumor recurrence
Chest CT (lung
windows) x Recurrence in 10% of patients
demonstrates
a pleural mass Malignant Mesothelioma
that forms x Most common primary pleural neoplasm
obtuse angles x JKKK

XKKK

$
at its interface
x 10% of exposed individuals
with the chest
wall. x $!

x Sixth to eighth decades
x

X

<
x Amphiboles most tumorigenic
x ]
XK_K

Figure 3 Malignant Mesothelioma: Clinical

x Insidious onset of symptoms
Localized
Fibrous Tumor. x 6-8 months prior to Dx
PA chest x Dyspnea, chest pain, cough, weight loss
radiograph x
$j
$
#
$
demonstrates a dysphagia, vocal cord paralysis, HPO,

large mass that

clubbing, hypoglycemia
of the right
hemithorax and Malignant Mesothelioma - Microscopic
produces mass x Epithelioid 50%
effect with x Sarcomatous 15%
contralateral x Biphasic 25%
shift of the
mediastinum. x Interobserver agreement 50%
Malignant Mesothelioma: Gross

x Sheets, plaques, masses
x Parietal > Visceral
Localized Fibrous Tumor: CT x Bulk in inferior hemithorax
[Figure 4] x Lung encasement
x
!
x Fissural growth
x Abutting pleural surface x Parenchymal involvement
x Elongated, lenticular x Mediastinal, chest wall, diaphragmatic
x #
invasion
¾Hemorrhage, necrosis, cysts
x Contrast enhancement Malignant Mesothelioma: Radiographic
[Figure 5]
x Pleural effusion
x Pleural masses
x Circumferential
Figure 4 x Mediastinal shift
x Pleural plaques 25%
Localized
Fibrous Tumor.
Contrast Figure 5
enhanced
chest CT Malignant
demonstrates Mesothelioma.
a large PA chest
heterogeneous radiograph
mass in the demonstrates
right lower circumferential,
hemithorax nodular and
with lobulated contiguous
contours. pleural masses
throughout the
left hemithorax.

Malignant Mesothelioma: Radiographic Malignant Mesothelioma: Treatment and
x Malignant mesothelioma cannot be Prognosis
reliably differentiated from pleural x
::
<K
!
metastases. x [

¾25-30% 5-year survival

Malignant Mesothelioma: CT ¾Negative margins
[Figure 6] ¾Epithelial cell type
x Pleural thickening 92% ¾No metastases
x Fissural thickening 86% x Extrapleural pneumonectomy
x Pleural effusion 74% ¾# !

x Ipsilateral È volume 42%
x

JK Pleural Metastases - Most Common
x Ipsilateral Ç volume 14% Pleural Neoplasm
Kawashima AJR 1990. x Common
¾Adenocarcinoma
Lung, breast, ovary, stomach
x Less common
¾Lymphoma, Invasive thymoma
x Imaging
¾Pleural effusion
¾Pleural masses
¾Or both
Pleural Metastases
x #

] !
x Direct extension
¾Lung Ca, Breast Ca
x Drop metastases
¾Invasive thymoma
x May be bilateral
Malignant Pleural Thickening

[Figure 7]
x Circumferential
x Nodular
¾> 1 cm
Figure 6 x Mediastinal pleura
Leung, et al. AJR 1990.
Chest CT demonstrates circumferential, nodular
pleural thickening in the left hemithorax that extends

"
plaques are demonstrated bilaterally.
Figure 7
Pleural
Metastases.
Malignant Mesothelioma: DX
x j
!

Chest CT
Sensitivity 98% demonstrates
¾

adenocarcinoma
entry ports arising from
a right upper
x

*!
!

lobe bronchus
VATS and pleural
x Cytology and FNA Bx of limited value metastases that
are nodular,
Malignant Mesothelioma: MR circumferential,
and involve
x Staging the mediastinal
x

/ pleura.
x Tumor enhancement
x Increased signal intensity

Chest Wall Chest Wall: I
' Infectious
x Congenital and developmental anomalies Diseases
x '

+
x Hematogenous
x Non-neoplastic conditions x Direct extension
x

x
+
S. aureus, P.
aeruginosa
Chest Wall: Congenital / Developmental x Imaging
Anomalies [Figures 8 and 9] ¾Osseous destruction if advanced
x Pectus deformities ¾/

+

x Anomalous ribs ¾/

+

x Cleidocranial dysostosis
x Poland syndrome Chest Wall: Tuberculosis [Figure 10]
x Uncommon
x Hematogenous spread
x Contiguous spread
x

J`
x Imaging
Figure 8 ¾[

¾Soft-tissue mass
Lateral chest ¾
radiograph ¾Peripheral enhancement

demonstrates
pectus
carinatum.
Figure 10
Tuberculosis.
Contrast enhanced chest CT demonstrates an anterior
chest wall abscess with subtle peripheral enhancement
and right pleural effusion and pleural thickening.
Chest Wall: I
' Infectious
Diseases
Figure 9
Chest Wall: Actinomycosis
Poland Syndrome.
Chest CT demonstrates congenital absence of the right [Figure 11]
pectoralis muscles. x Actinomyces israelii
x Anaerobic gram-positive
x Lung ĺPleura ĺChest Wall
x Proteolysis ĺFistulas
x ~

x '
+

periostitis

Chest Wall Neoplams: Soft Tissue
Involvement [Figure 12]
x Adults
x
¾Fibrosarcoma
¾

!
Figure 12
Figure 11
Fibrosarcoma.
Actinomycosis. Chest CT demonstrates post-surgical changes of right
Contrast enhanced chest CT demonstrates peripheral mastectomy and a soft-tissue mass in the left chest
consolidation in the left upper lobe with contiguous wall. The patient received radiation therapy through
soft-tissue density extending into the adjacent ports that included the left posterolateral chest wall.
mediastinum and anterior chest wall.
Chest Wall: Neoplasms

x Adults
x [
¾Lipoma
¾Other mesenchymal neoplasms
x
¾Fibrosarcoma
¾

!
¾Other mesenchymal neoplasms
¾Lymphoma
Chest Wall Neoplasms: Lipoma

x Common
x Subcutaneous
x Intrathoracic
x Both
x Diagnostic CT number
Chest Wall Neoplasms: Desmoid

(Fibromatosis)
x :

x

+!

x Shoulder, chest wall
x Soft tissue mass
x Frequent recurrence

Chest Wall Neoplasms: Osseous Chest Wall Neoplasms: Chondrosarcoma
Involvement [Figure 13] x Adults
x Rib expansion x Painful, palpable mass
¾Fibrous dysplasia x Costochondral junction, rib, sternum
¾Enchondroma x '
x Pressure erosion ¾Expansile, destructive

¾Neurogenic (slow growth) ¾!

x Rib destruction ¾Soft-tissue mass
¾Metastatic or Primary
¾' PNET “Askin Tumor”: Primitive
Neuroectodermal Tumor
x Malignant small round cell tumor
x Children, adolescents
x

X<
x Unilateral
x

JX
Figure 13 x Pleural effusion
x Poor prognosis
Fibrous
Dysplasia.
Chest CT
demonstrates
an expansile
lesion with
intact cortical
margins
involving a
right rib.
Pressure Erosion
x Slow growth
x Neurogenic
Chest Wall Neoplasms: Osseous

Destruction
x Adult
¾Metastatic disease (Lung, Breast,
Prostate, etc.)
¾Multiple myeloma
¾Chondrosarcoma
x Child
¾Ewing sarcoma
¾Neuroblastoma
¾Lymphoma
¾Askin tumor (PPNET)
Chest Wall Neoplasms: Myeloma

x Males > Females
x 5th - 7th decades
x Axial skeleton
x Multiple or solitary
x '
¾Osseous destruction
¾Soft-tissue mass

(AIRP 2010) Vol. 1 Chest

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Chest Radiology

Describing Diffuse Lung Disease

Chronic diseases tend

An Approach to Diffuse Lung Disease

An Approach to Diffuse Lung Disease 12 Chest Radiology

Anatomy - Secondary Lobule

Chest Radiology 13 An Approach to Diffuse Lung Disease

sources of 1-25 Dihydroxyvitamin

Sarcoidosis: Clinical Features

Non-Caseating Granuloma and Fibrosis

An Approach to Diffuse Lung Disease 14 Chest Radiology

Sarcoidosis: Computed Tomography

Sarcoidosis: Staging Based on

Chest Radiology 15 An Approach to Diffuse Lung Disease

Extrinsic Allergic Alveolitis

Sarcoidosis: Cardiac Involvement

Ground Glass Opacities

Consolidation and Large Nodules

Bronchovascular Bundle Distortion

Fibrosis and Emphysema

Fibrosis and Honeycombing

An Approach to Diffuse Lung Disease 16 Chest Radiology

Chest Radiology 17 An Approach to Diffuse Lung Disease

An Approach to Diffuse Lung Disease 18 Chest Radiology

Chest Radiology 19 An Approach to Diffuse Lung Disease

¾Weeks to months x Idiopathic Pulmonary Fibrosis (IPF)

involve the pattern

dust, metal dust

The lung Usual Interstitial Pneumonia: Histology

IPF-Progressive Volume Loss

Smoking Related ILD - RB

Chest Radiology 23 

Smoking Related ILD [Figure 8]

Acute Interstitial Pneumonia:

Cryptogenic Organizing Pneumonia

Chest Radiology 25 

Cryptogenic Organizing Pneumonia:

/     < 

Cryptogenic Organizing Pneumonia

NSIP ATS Consensus Conference Figure 15

NSIP Fibrosis with IPF Imaging

Katzenstein AA, et al. Amer J of Surg Path 2002.

NSIP in Cigarette Smokers

OP-NSIP [Figure 15]

The Idiopathic Interstitial Pneumonias:

Chest Radiology 27 

#" Organizing Pneumonia.

Acute Interstitial Pneumonia NSIP in the Literature

NSIP-Cigarette Smokers [Figure 22]

NSIP-Organizing Pneumonia [Figure 23]

Patients with organizing pneumonia may have a biopsy

Chest Radiology 29 

Differential Diagnosis of Airways Small airway

Diseases with Obstructive Physiology

The Changing Role of Imaging

Why Pulmonary Functions are

Diffuse Lung Disease - Airways [Figure 3]

Airways Disease 34 Chest Radiology

Airways Disease - Indirect Signs

Chest Radiology 35 Airways Disease

Emphysema and Computed Tomography

Relationship of RB and Emphysema

Nagai & Thurlbeck. Am Rev Respir Dis 1985.

Chest Radiology 23

Chest Radiology 25

/ <

Chest Radiology 27

#" Organizing Pneumonia.

Chest Radiology 29

Alpha-1 Antitrypsin D

Alpha-1 Antitrypsin D

Constrictive Bronchiolitis - C

%>/

%* / /

Alpha-1 Antitrypsin D

/%*

Chest Radiology 53

Chest Radiology 55