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Mediastinal Tumors

BY TEMESGEN G/MARIAM
OUT LINE

ANATOMY
CONTENTS
PATHOLOGIC ENTITIES
CLINICAL PRESENTATION
DIAGNOSTIC EVALUATION
TUMORS
CYSTS
Anatomy
PATHOLOGIC ENTITIES
ASM MM PS

-Thymoma -Cysts-bronchogenic -Neurogenic Tumors


- Lymphoma - pleuropericardial -Neuroenteric cysts

- Germ cell tumors -enterogenous -lymphoma


-Abberant thyroid -Lymphoma

- Lipoma - Thoracic duct cyst


-Parathyroid adenoma - Hamartoma
-Thymic cyst - Mesenchymal tumors
-Haemangioma
-Lymphangioma &cysts
-Fibroma
-Fibrosarcoma
Overall mediastinal tumors are rare
The most common tumors are:
neurogenic tumors(20%)

thymoma(19%)

primary cysts(18%)

lymphomas(13%)

GCT(10%)

Most are located in ASM (54%), followed by PM (26%)


and MM (20%)in adults
25% of mediastinal tumors are malignant
Anteriosuperior 59%

Posterior 16%

Middle 25%

95% of incidentally found tumors are benign


Symptomatic lesions are benign, malignant
Adults
Children
Anterior Mediastinum
Posterior Mediastinum
Often Malignant
Most often benign
Ages 30 50
2/3 of tumors are
1/3 of tumors are symptomatic
symptomatic

CLINICAL PRESENTATION
Most common Symptoms include
Chest pain

Dyspnea

Cough

Fever

Other: Wt loss, fatigue, sweating, hemoptysis , stridor


Compressive sxs

SVS
Pericardial tamponade
CHF
Chylothorax
Rhythm disturbance
Vocal cord paralysis
Horners syndrome
Phrenic nerve paralysis
Spinal cord compression
Pancoasts syndrome
pulmonary stenosis
Dysphagia
Stridor
Paraneoplastic syndrome

Hypertensionpheochromocytoma
Hypercalcemiaparathyroid tumor
Thyrotoxicosis intrathoracic goiter
Gynecomastia choriocarcinoma
Pel-Ebsten fever Hodgkins disease
Myastenia gravis thymoma
Ostiodystrophy neurogenic tumors
Diarhea neuroblastoma
DIAGNOTIC EVALUATION

History
P/E
General- chronically sick, Cushinoid face, in distress
Ptosis
Sign of SVS
Flaring, audible stridor, retractions, palpable chest masses
through the thoracic inlet
Sign of effusion, collapse
Sign of CHF
HM, Abdomenal mass
Limitation of movement, vertebral mass, vertebral deformity
Smymptx/sign of spinal compression
Skin lesions
Investigations
CBC, U/A, OFT
Imaging techniques
CXR (PA & lateral)

Ultrasound

Barium swallow

Echocardiography

CT scan

MRI

Radionucleotide studies: 131I Radioiodine scan, Technetium-


99m-sestamibi
131I metaiodobenzylguanidine scintigraphy Gallium
scintigraphy
NONSURGICAL BIOPSIES :
Percutanious biopsies

FNA

Core-needle biopsy

CT guided needle biopsy

SURGICAL BIOPSIES & RESECTION:


Mediastinoscopy- paratrachial masses

VATS- anterior or posterior med. Masses

Anterior mediastinotomy-anterior tumor or significant


parasternal extension
Bronchscopy

Oesophagoscopy

Thoracotomy
Biochemical markers
VMA

Metanephrine- Pheochromocytoma

Alpha fetoprtein

Beta hCG- NSGCT

ACTH- Carcinoid tumors

T3,T4, TSH- toxic substernal goiter

Serum Ca- Parathyroid adenoma


THYMUS

THYMOMA
Most common anterior mediastinal neoplasm in adults
(31%)
2nd most common mediastinal tumor (19%)

40-60 yrs of age

Rare in children
C/F
Asymptomatic (40-60%)

Mass effects

10-50%--MG sxs

5%--other paraneoplastic syndromes

In case of malignant/invasive thymoma

pleural and pericardial effusion

Wt loss, fatigue, fever, night sweating


Diagnosis

Hx & P/E : suspect diagnosis


Investigation
- Basic routine
CXR(lateral)- smooth small, well-circumscribed mass or bulky
lobulated mass confluent with adjacent mediastinal structures
calcification 10%(usually peripheral & linear)
CT, MRI:- homogeneous, heterogeneous, calcification
Mediastinoscopy
CT guided FNA-sn=87%,sp=95%
Cytokeratin-to distinguish it from lymphoma
Microscopic classification

Lymphoepithelial (50%)
Epithelial (27%)
Lymphocytic (22%)
spindle cell variety, a subtype of epithelial thymoma
Cell types as cortical, medullary, or mixed on the
basis of immunohistochemistry and light
microscopy
May be malignant(3060%) or benign(40-70%)
Benign no capsular invasion
Degenerative changes- hemorrhage, calcification,
necrosis, cystic changes
Pathological staging-(Masaoka)
Based on:
Invasion of capsule & surrounding stractures

Metastasis
Stage 1-encapsulated,no invasion
Stage 2-capsular invasion-mediastinal fat,pleura
Stage 3-gross invasion-pericardium,lung,great vss
Stage 4-a-pleural or pericardial dissemination
b-metastasis (lymphatic / hematogenous)
Rx.
Stage I purely surgery

Stage II + III Surgery +Radiotherapy

Stage IV aggressive multimodal therapy

Debulking + RRx +
Cisplastin based chemotherapy
Prognosis--- 5 Yr survival
Stage I 85-100%

Stage II 60-80%

Stage III 40-60%

Stage IV 50%

Thymomas frequently show recurrence, and


reoperation for recurrent disease has been
recommended
THYMIC HYPERPLASIA
1st described in children after chemotherapy
In adults-rebound thymic hyperplasia
9 mo after chemotherapy cessation
Needs careful follow up
serial CT scans

PET scan

biopsy-high index of suspicion


THYMIC CARCINOMA

Malignant at the microscopic level


Rare
Age- 46-54yrs sex- M>F
C/F:-chest symptoms , general symptoms
not associated with paraneoplastic syndromes

<10% are asymptomatic

Ix: CXR/CT- infiltration , necrosis , calcification


Low grade- SCC, mucoepidermoid,or basaloid
High grade- Lymphoepithelial-like.,small-cell,
sarcomatoid, clear cell
Early local invasion & wide spread metastasis so with
poor prognosis
Rx:
Complete resection

Occasionaly curative

Most recure & are refractory to chemotherapy

Radiotherapy
Neoadjuvant therapy
Thymolipomas

Rare benign tumors


Well encapsulated, soft,
and pliable masses
Resection is
recommended for large
masses
MEDIASTINAL GERM CELL TUMORS

most frequent location for extragonadal GCT.


Most are gonadal in origin. But mediastinum as the primary
site are rare, < 5% of all germ cell tumors
11% of all mediastinal tumors are GCT
young male patients in their 3rd decade
anterior compartment
1/3 seminomatous
Ix:-CT-multilocular cystic tumors
FNA biopsy-
serum markers-
core needle biopsy-
surgical biopsy
Classification of Germ Cell Tumors

Benign
Mature teratomas

Dermoid cysts

Malignant
Seminomas
Nonseminomatous germ cell tumors
Immature teratoma
Teratoma with malignant components
Choriocarcinomas
Embryonal cell carcinomas
Endodermal cell (yolk sac) tumors
Mixed germ cell tumors
TERATOMA
Most common type of med.GCTs(60-70%)
2 or 3 embryonic layers, skin, hair (ectodermal),
cartilage and bone (mesodermal), or bronchial,
intestinal, or pancreatic tissue (endodermal).
Peak incidence is in the second and third decades of
life.
No gender predisposition.
Most in the ASM, although 3% to 8% PM
Rarely, focus of carcinoma;these malignant
teratomas(or teratocarcinomas) are locally aggressive
characteristic CT findings of a multilocular cystic
tumor, encapsulated with combinations of fluid, soft
tissue, calcium, and/or fat attenuation in the anterior
compartment
Rx-surgical resection for benign
Treatment with cisplatin-based chemotherapy is
instituted for malignant
Poor prognosis for malignant even after treatment
5-year survival rate of 61 %for patients responding to
chemotherapy and < 10% for non-responders has
been reported
SEMINOMAS:
50% of malignant germ cell tumors and 2% to 4% of all
mediastinal masses.
large cells with round nuclei, scant cytoplasm, and
abundant glycogen.
Advanced disease at time of diagnosis
Local compression than metastasis
Rx-Radiation therapy best response (90%)
Surgical resection- small asymptomatic or residual after
chemotherapy
chemotherapy-cisplatin-based
75%-complete response
NONSEMINOMATOUS GCTS
Bulky,irregular tumors
Involvment of adjacent stractures
Metastasis to regional LNs,pleura,& lungs,brain
s/mic Children with these tumors may present with
precocious puberty
chromosomal abnormalities are associated
Ix -CT- areas of low attenuation
-Serum markers(LDH,AFP,hCG)
Rx -chemotherapy
Cisplatin,bleomycin,etoposide
2yr survival=67%,5yr survival=60%
surgical resection of residual mass/salvage chemotherapy
rare radiosensitivity
NEUROGENIC TUMORS
Arise from nerve sheath cells,ganglion cells,or
paraganglionic system; vagus & phrenic nerves
Commonest tumor in the mediastinum
Commonly arises from posterior mediastinum
Age-correlates with incidence , cell type,& malignancy
risk
In children, most neurogenic tumors are malignant
(50%)
Rate of malignancy low in adults (10%)
Could have intraspinal extension=>Cord
compression
Chest pain, dyspnea, hoarse voice
Horners syndrome - unusual
Classification
NERVE SHEATH TUMORS
20% of all mediastinal tumors
>95%-benign(neurilemmoma or neurofibroma)
NEURILEMMOMA(schwanomas)
Arise from intercostal nerves
Benign,firm,well encapsulated
Malig. Tumors lack encapsulation & no Antoni features
Dx:
Hx

Ix-CT,MRI: firm, well-encapsulated, and generally benign

FNAC: Antoni type A compact spindle cells with twisted nuclei


and nuclear palisading. Antoni type B regions contain loose and
myxoid connective tissue with a haphazard cellular arrangement
Rx:
Conservative-small, asymptomatic paravertebral tumors,older
patients
Resection-open thoracotomy

VATS

Complete resection
Combined thoracic and neurosurgical approach is
indicated for tumors with intraspinal extension
Recurrence is rare for benign tumors
Local recurrence is common for malignant tumors
and overall prognosis is poor
NEUROFIBROMA
Both nerve sheath & nerve cell components
25% of nerve sheath tumors
70 % -benign
Malignant degeneration(25-30%)
Risk - with age, vRh disease, radiation exposure
Prognosis- growth rate , local invasion along nerve
bundles
Rx-complete resection
5yr survival rate-53%
GANGLION CELL TUMORS
From sympathetic chain or adrenal medulla
GANGLIONEUROMA:
Well differentiated cystic degeneration
Asymptomatic, young adults & childhood
Intraspinal canal extension
Rx-complete resection

GANGLIONEUROBLASTOMA:
mature and immature ganglion cells.
Nodular-metastasizes
Diffuse-rarely metastasizes
Infants & children<3yrs
Rx-resection & chemotherapy.
5yr servival=80%
NEUROBLASTOMA

Highly malignant
Most common intrathoracic malignancy of childhood
Adrenal gland-common site but 14% arise from thorax
spontaneously regress, mature, or proliferate
aggressively
Extension in to spinal canal & ossious invasion
Children <2 yrs(>50%)-
Rx-surgical resection
chemotherapy
better prognosis than neuroblastomas occurring
elsewhere
PARAGANGLIONIC TUMORS
PHEOCHROMOCYTOMA:
one of the most rare tumors
10% are malignant
Highly vascular
Site-costovertebral sulcus
-visceral compartment
Preoperative care includes alpha- and beta-adrenergic
blockade to prevent intraoperative malignant hypertension
and arrhythmias

CHEMODECTOMA:
Rare
30% malignant, rarely secrete catecholamines
Site-aortic arch,vagus,aorticosympathetic
MEDIASTINAL CYSTS
PRIMARY MEDIASTINAL CYST
25% of med.masses are benign cysts

Middle compartment

CT-near water density in a typical location


BRONCHOGENIC CYST
From mediastinum & pulm. parenchyma(15%) usually
subcarinal area
Children-most are symptomatic
-serious complications
Adults-> found incidentally
Sxs- chest pain, cough, dypnoea & fever
Complications are not common but
Malignant degeneration has been repoted
Dx
CXR ovoid, tear drop, with calcification, air-fluid level

Bronchoscopy

Ba study

CT, MRI

Rx-
Resection

Thoracoscopic exploration & resection by VATS &

Incomplete resection by mediastinoscopy

Aspiration & excision


PERICARDIAL CYST
The most common type of mediastinal cysts
Asymptomatic
100% benign
Rt costophrenic angle
Dx __ CXR, CT, MRI
Rx
-observation
-aspiration
-surgical resection
ENTERIC CYST

Propensity for serious complications


Cxs- hemorrhage, infection, perforation
Rx- surgical resection
REFERENCES

Schwatzs principles of surgery 9th ed.


Oxford textbook of surgery 2nd ed.
Thomas ,general thoracic surgery 4th ed.
Up to date 17.1