INFLAMMATORY PSEUDOTUMORONLINE
(PLASMAEXCLUSIVE
CELL GRANULOMA) OF THE TEMPORAL BONE
Inflammatory pseudotumor (plasma
cell granuloma) of the temporal bone
Dare V. Ajibade, BA; Iwao K. Tanaka, MD; Kapila V. Paghda, PharmD, MD;
Neena Mirani, MD; Huey-Jen Lee, MD; Robert W. Jyung, MD
Abstract
We report the case of a 41-year-old man who presented
with progressive right-sided ear pressure, otalgia, hear-
ing loss, tinnitus, and intermittent otorrhea. Computed
tomography and magnetic resonance imaging detected a
soft-tissue mass in the right mastoid with intracranial inva-
sion and erosion through the tegmen tympani and mastoid
cortex. Histopathologic examination was consistent with
an inflammatory pseudotumor (plasma cell granuloma).
These lesions rarely occur in the temporal bone. When they
do, they are locally destructive and can erode bone and
soft tissues. Aggressive surgery is recommended as a first-
line treatment, with adjunctive steroid or radiotherapy
reserved for residual or refractory disease. Our patient
subsequently experienced multiple recurrences, and his
treatment required all of these modalities. At the most
recent follow-up, he was disease-free and doing well.
Introduction
An inflammatory pseudotumor is an unencapsulated,
benign tumor of unclear etiology. It was first described
by Bahadori and Liebow in 1973.1 This uncommon
inflammatory lesion has also been referred to as a
plasma cell granuloma, inflammatory myofibroblastic
pseudotumor, inflammatory histiocytoma, and inflam-
matory fibrosarcoma. It most commonly arises in the
lungs, but it has been reported in nearly every soft-tissue
structure of the body.
Inflammatory pseudotumors of the head and neck
From the Division of Otolaryngology–Head and Neck Surgery, Department
of Surgery (Mr. Ajibade, Dr. Tanaka, Dr. Paghda, and Dr. Jyung),
the Department of Pathology (Dr. Mirani), and the Department
of Radiology (Dr. Lee), New Jersey Medical School, University of
Medicine and Dentistry of New Jersey, Newark.
Corresponding author: Robert W. Jyung, MD, 140 Bergen St., ACC Suite
E1620, Newark, NJ 07103. E-mail: jyungrw@umdnj.edu
Volume 89, Number 7
are rare, accounting for fewer than 5% of all extrapul-
monary cases.2 In the head and neck, the most com-
mon location is the orbit, followed by the meninges,
paranasal sinuses, infratemporal fossa, and soft tissues.
The temporal bone, skull base, and facial nerve are very
rarely involved. Indeed, our review of the literature
revealed only 35 previous case reports of inflammatory
pseudotumor affecting the skull base and the temporal
bone area (table 1).3-23 The most common presenting
symptoms of tumor growth resulting in local destruc-
tion of middle ear structures are otalgia, hearing loss,
and otorrhea. Standard treatments include surgical
excision, steroid therapy, and radiotherapy, depending
on the size and location of the tumor, as well as the
patient’s age and comorbidities.7,24
In this article, we report a new case of inflammatory
pseudotumor of the temporal bone, and we review the
literature on this rare finding.
Case report
A 41-year-old man of South Asian descent was re-
ferred to the senior author (R.W.J.) for evaluation of a
7-month history of right-sided ear pressure and pain,
hearing loss, tinnitus, and intermittent drainage. The
pain was sharp; it extended from the ear and mastoid
area and radiated down the right side of the neck. The
patient denied vertigo or imbalance, and his left ear
was uninvolved.
The referring otolaryngologist had performed a right
myringotomy 3 months earlier, and it had revealed the
presence of a thick, cloudy fluid that grew normal ear
flora on culture. Postoperatively, the patient reported
the onset of pulsatile tinnitus.
At the referral visit, the patient reported no history
of other ear surgeries, relevant trauma, recurrent infec-
tions, or exposure to excessive noise. He did report that
he had traveled to Hong Kong four times during the
previous year. On physical examination, the external
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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG

Table 1. Summary of reported cases of inflammatory pseudotumor involving the temporal bone

Age/ Facial nerve

Author sex Site Presenting symptom involvement

Yanagihara et al,3 41/M Geniculate ganglion Right facial palsy Yes

1991

Benton et al,4 1992 27/M Mastoid, middle cranial Otalgia Yes

fossa, and petrous apex

Nam et al,5 1994 24/F Mastoid Otalgia and hearing loss Not reported

Wiseman et al,6 1995 4/M Adjacent to the vertical Otitis media and Yes
portion of cranial nerve VII complete left facial palsy

Mulder et al,7 1995 39/M Tympanic cavity, mastoid, Hearing loss, otalgia, Yes
and internal auditory canal and vertigo

38/F Internal auditory canal, Otorrhea following Yes

cochlea, middle ear, six operations for
and mastoid cholesteatoma

50/F Tympanic cavity and Chronic otitis with otorrhea, Not reported
mastoid otalgia, and hearing loss

Janicki et al,8 1996 55/M Mastoid, middle ear, Otorrhea, tinnitus, No

posterior fossa dura, and vertigo
and petrous apex

Lee HM et al,9 2001 64/F Right maxillary sinus Right nasal obstruction No
and several episodes
of epistaxis

Williamson et al,10 49/M Mastoid attic, tegmen Hearing loss and otorrhea No
2003 tympani (middle cranial
fossa), epitympanum,
eustachian tube, lateral
and superior semicircular
canals

Gasparotti et al,11 26/M Mastoid attic, dura, Vertigo Yes

2003 and tentorium

Cho AH et al,12 2004 42/F Mastoid, petrous bone, Headache, tinnitus, hearing Yes
middle ear, dura, tentorium, loss, right facial palsy,
and cavernous sinus diplopia, and visual dimness

Lee DK et al,13 2006 62/M Skull base and cervical Headache, right temporo- No
vertebra mandibular joint pain, right
hearing loss, and right
middle-ear effusion

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 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE

Follow-up
Treatment and outcome

Excision 25% cranial nerve VII

function at 2 yr

Craniotomy, radical No evidence of disease

mastoidectomy, and on physical examination
radiotherapy and MRI at 1 yr

Canal-wall-down No evidence of
mastoidectomy and disease at 1 yr
radiotherapy

Excision 100% cranial nerve VII

function at 1 mo

Transotic excision No complaints at 2.5 yr

Transotic excision No complaints at 1 yr

Subtotal petrosectomy No complaints at 9 mo

Simple mastoidectomy Persistent mass;

and radiotherapy no increase in
size on MRI at 12 mo

Excision No evidence of
recurrence at 24 mo

Tympanomastoidectomy, Significant decrease in

subtotal petrosectomy, dural and temporal lobe
and steroids enhancement on MRI
at 6 mo

Extended mastoidectomy Residual dural

enhancement on
CT on postoperative
day 1

Steroids Marked decrease

in mass size in the
right temporal bone,
cavernous sinus, and
meninges on MRI at 3 mo

Steroids and radiotherapy Headache improvement

and normal erythrocyte
sedimentation rate
(ESR) at 13 mo

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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG

Table 1. continued.

Age/ Facial nerve

Author sex Site Presenting symptom involvement

Lee DK et al,13 2006 61/F Skull base Headache and left eye pain No
cont.

64/F Skull base Headache, hoarseness, No

dysphagia, and right
hearing loss

52/F Skull base Bilateral facial palsy and Yes

left facial numbness

69/F Skull base Headache, right ptosis, Yes

vision loss, and right facial
nerve palsy

76/M Skull base Headache, weight loss, No

and right otorrhea

63/M Skull base Headache, dysphagia, No

and hoarseness

52/M Skull base Headache and left No

facial numbness

McCall et al,14 2006 28/M Cavernous sinus and Headache, nausea, No

skull base dizziness, double vision,
and VIth cranial nerve palsy

Cho KJ et al,15 2007 55/F Middle ear and mastoid Hearing loss and otorrhea No

Lee RG et al,16 2007 28/F Middle ear Progressive left facial Yes
weakness and intermittent
mild left otalgia

Diaz et al,17 2007 53/F Right endolymphatic sac, Right hearing loss, tinnitus, No
vestibule, and semicircular otalgia, vertigo, and
canals imbalance

41/F Right endolymphatic sac, Right aural fullness No

posterior fossa dura,
and sigmoid sinus

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 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE

Follow-up
Treatment and outcome

Steroid taper and Headache improvement

radiotherapy and normal ESR
at 24 mo

High-dose steroid taper Headache improvement

and normal ESR
at 10 mo

Steroid taper Headache improvement

and normal ESR
at 16 mo

Steroid taper and Headache improvement

radiotherapy and normal ESR
at 9 mo

Steroid taper and Headache improvement

radiotherapy and normal ESR
at 3 mo

Steroid taper and Headache improvement

radiotherapy and normal ESR
at 3 mo

Steroid taper and Headache improvement

radiotherapy and normal ESR
at 6 mo

Empiric warfarin, steroids, Near complete

and ceftriaxone for a resolution on MRI 1 yr
presumed sinus after biopsy
thrombosis secondary to
an infectious process, and
subsequent steroid taper

Canal-wall-down No evidence of recurrence

mastoidectomy 1 yr postoperatively

Transcanal and Improved facial nerve

transmastoid excision function from House-
Brackmann grade III to VI
and no tumor recurrence
at 3 yr

Translabyrinthine resection Disease-free 23 mo

with skeletonization of the post-treatment
internal auditory canal

Transjugular approach Disease-free 26 mo

with resection of the post-treatment
posterior fossa dura and
sigmoid sinus

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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG
Table 1. continued
Age/
Author sex Site
Lee JH et al,18 2007 39/M Right middle ear, mastoid
antrum, and left lung
Strasnick and 65/F Right temporal bone
Vaughan,19 2008 with extension into the
middle cranial fossa
40/M Right petrous apex, right
sphenoid bone, cavernous
sinus, and pterygopalatine
fossa
Santaolalla-Montoya 75/M Temporal bone with
et al,20 2008 skull base extension
Coulson et al,21 2008 60/M Right middle ear and
mastoid, skull base,
and temporal and parietal
brain parenchyma
Allona et al,22 2009 28/M Left petrous apex, along
with thrombosis of the
distal sigmoid sinus
Garg et al,23 2010 54/F Infiltrating skull-base
lesion extending into the
jugular fossa, clivus, and
nasopharynx, and encasing
the left carotid artery
46/F Central and right
nasopharynx, encasing
the right internal carotid
artery
61/F Nasopharyngeal and
infratemporal fossa,
encasing the right internal
carotid artery
72/F Infratemporal mass
extending into the foramen
rotundum and the maxillary
sinus and involving the
pterygopalatine fossa,
temporalis, masseter,
and parotid
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Facial nerve
Presenting symptom involvement
Hearing loss, retrobulbar No
pain, and diplopia secondary
to right VIth nerve palsy
Right hearing loss, No
dizziness, tinnitus, and
complex partial seizures
Right facial pain, aural No
fullness, and hearing loss
Severe headache, Yes
intermittent right-sided
otorrhea, and hearing loss
Right hearing loss Yes
followed by otorrhea and
otalgia after tube insertion
Cephalea and seizures No
Left otalgia, aural fullness, No
sore throat, and temporo-
occipital headaches
Right frontal headache, No
V2 numbness, and
VIth nerve palsy
Right otalgia No
Pain in the left maxilla No
and mandible, trismus,
weight loss, dysphagia,
and nasal congestion
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 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE

Follow-up
Treatment and outcome

Mastoidectomy for biopsy Asymptomatic 1 yr

and steroids post-treatment

Transmastoid and middle Seizure resolution at 8 yr

cranial fossa approach for
resection with postoperative
radiotherapy and steroids

Right mastoidectomy and No recurrence at 10 yr

middle fossa craniotomy
with postoperative
radiotherapy

High-dose intravenous Death 6 mo after initial

ceftazidime at 2 g/8 hr, presentation
oral prednisolone at
30 mg/d, and carbamazepine
at 200 mg/12 hr for 6 wk

Transmastoid biopsy and Symptom-free 18 mo

steroids after initial treatment

Biopsy Spontaneous partial

regression

Prednisone at 5 mg/day, Asymptomatic at 3 mo;

lowered to 2.5 mg/day stable appearance of the
after 3.5 yr sclerotic process on MRI
at 2 yr; complete resolution
on MRI at 3.5 yr

Prednisone at 60 mg/day Headache resolution with

normal vision at 2 mo;
marked decrease in mass
size on MRI and normal
endoscopy at 12 mo

Prednisone at 10 mg/day, Significant reduction

decreased to 5 mg/day in size at 1yr on MRI;
complete symptom
resolution at 18 mo

Prednisone at 40 mg/day, Symptom improvement

lowered to 15 mg/day at 1 yr; resolution of the
over 6 mo and to mass on CT at 2 yr
10 mg/day after 1 yr

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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG
Table 1. Continued
Age/
Author sex Site
Ajibade et al,* 2010 41/M Right mastoid with focal
erosion through the
tegmen tympani and
mastoid cortex
* Present case.
ears were normal bilaterally. An area of point tenderness
was noted above the right pinna, and an erythematous,
nonfluctuant swelling was detected over the right
mastoid and neck. The right external auditory canal
was tender and exhibited significant posterosuperior
skin thickening, which obscured the right tympanic
membrane; the tympanic membrane was opaque and
thickened. The Weber test lateralized to the right, and
the Rinne test was negative on the right; these findings
were consistent with a right-sided conductive hearing
loss. However, audiometry demonstrated a mild high-
frequency sensorineural hearing loss on the right with
no conductive deficit, and a type C right tympanogram
suggested eustachian tube dysfunction.
High-resolution computed tomography (CT) re-
vealed that a soft-tissue mass had filled the right mas-
toid and focally eroded through the tegmen tympani
and mastoid cortex. Contrast-enhanced magnetic
resonance imaging (MRI) demonstrated a diffuse,
homogeneous lesion in the right mastoid (figure 1).
Figure 1. T1-weighted MRI with gadolinium contrast shows a mild
homogeneous enhancement of the mass in the right mastoid. No
dural enhancement or thickening is seen.
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Facial nerve
Presenting symptom involvement
Right aural pressure, No
otalgia, hearing loss,
tinnitus, and intermittent
drainage
The patient underwent a right canal-wall-up mas-
toidectomy with diagnostic and therapeutic intent.
Surgical exposure revealed very adherent, fibrous,
and rubbery vascular tissue that extended through
multiple mastoid air cell tracts, including the mastoid
tip, and eroded through the tegmen tympani and
mastoid cortex, with invasion of the medial aspect of
the temporalis muscle.
Intraoperative frozen-section analysis revealed “lym-
phoblastic infiltrate.” Therefore, the decision was made
to end the procedure and to leave the residual tissue in
the mastoid antrum in the event that the final pathol-
ogy report established a diagnosis of lymphoma. The
final pathologic diagnosis was “plasma cell granuloma.”
Histology of the lesion revealed fibroconnective tissue
with a heavy cellular infiltrate composed of predomi-
nantly mature plasma cells with Russell bodies (figure
2), a small number of lymphocytes with follicles,
neutrophils, a few eosinophils, and fibrosis. Plasma
cells were immunoreactive to kappa and lambda light
chains, suggesting the polyclonal nature of the lesion.
In situ hybridization for Epstein-Barr virus (EBV)
was negative.
Prednisone at 60 mg/day followed by a taper was
initiated for the treatment of the residual inflamma-
tory pseudotumor. However, after 1 week of therapy,
the dose was reduced in response to complaints of
hip and lower-extremity pain, symptoms that are
associated with avascular necrosis.25 One month
postoperatively, the patient reported decreased pain
and pressure, continued tinnitus, and no change in
hearing. On physical examination, his right ear canal
was noticeably more patent, and otoscopy revealed a
thinner, translucent tympanic membrane with focal
areas of neovascularization.
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 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE

loss, and otorrhea might suggest chronic otitis media

or a cholesteatoma, he had a number of clinical and
Follow-up radiographic findings suggestive of a neoplasm. Limited
Treatment and outcome otoscopy revealed involvement of the external auditory
canal, but no visible cholesteatoma, retraction pocket,
Canal-wall-up Disease-free at last
or perforation was present. Audiometry showed no
mastoidectomy for biopsy follow-up conductive hearing loss, as would be expected with a
with postoperative steroids, cholesteatoma or chronic otitis media. Moreover, the
then definitive excision via focal area of bone destruction noted laterally through
revision canal-wall-up the mastoid cortex was atypical for cholesteatoma.
mastoidectomy, temporal
and middle fossa craniotomy, Instead, these findings were consistent with those de-
and duraplasty with postoperative scribed in other reports of temporal bone inflammatory
steroids and radiotherapy pseudotumors, including homogeneous enhancement
with contrast and areas of bony destruction along with
areas of radiographic normality; also, dural enhance-
Four months postoperatively, the patient reported ment has been described in many cases (table 2).7,11
that he had been feeling lethargic for the previous 3 The absence of middle ear involvement both on CT
weeks. A follow-up MRI detected an enhancing mass and MRI suggested a neoplastic lesion rather than a
in the right temporal bone that extended into the dura cholesteatoma or mastoiditis.
of the middle cranial fossa, with no midline shift. This Temporal bone inflammatory pseudotumors have
finding was consistent with a recurrence of the mass, and been successfully treated with surgical excision, ra-
surgical excision was therefore deemed to be the best diotherapy, and high-dose steroids, all of which we
option. A right revision canal-wall-up mastoidectomy, used in our patient. In view of the aggressive and
a temporal and right middle cranial fossa craniotomy, unpredictable course of temporal bone inflammatory
and a duraplasty were performed. Pathology confirmed pseudotumor, some authors believe that it warrants
that the mass represented a recurrence of the inflam- urgent evaluation with biopsy and definitive surgical
matory pseudotumor. Steroid therapy was instituted therapy.24 Because these tumors are unencapsulated,
postoperatively. surgical excision with wide margins is necessary to
Ten months following the second surgery, the patient prevent recurrence. A recurrence rate of 22% at 12
returned complaining of weakness in his left hand months following surgical excision has been reported.24
and difficulty speaking. Another MRI demonstrated Tumors that involve the facial nerve or skull base can
a focal area of enhancement in the right parietal area make complete excision difficult in view of the poten-
with considerable vasogenic edema in the surrounding
area. Given the absence of a mass, radiotherapy was
administered at a dose of 4,500 cGy in 25 fractions
over 36 days. At the most recent follow-up, he was
disease-free and doing well.

Discussion
Inflammatory pseudotumors usually demonstrate
unusual clinical, radiographic, and pathologic features.
In terms of etiology, infectious, autoimmune, and neo-
plastic processes have been proposed. Infections with
EBV or human herpesvirus 8 have been implicated in
inflammatory pseudotumors of the spleen and liver.7,24
Because our patient reported frequent travel to Hong
Figure 2. On H&E staining, the biopsy specimen exhibits dense
Kong, a region where EBV-associated neoplasms are
fibroconnective tissue with heavy lymphoplasmacytic cellular
more common, we examined his lesion for EBV DNA infiltrate composed of mature plasma cells and lymphocytes with
and found no evidence of EBV infection. follicles, but few eosinophils and neutrophils. Occasional Russell
While our patient’s complaints of otalgia, hearing bodies are seen.

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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG
Table 2. CT and MRI findings in selected cases
Author CT
Yanagihara et al,3 1991 Low-density shadow about the right geniculate
ganglion
Benton et al,4 1992 Not reported
Nam et al,5 1994 Soft-tissue mass in the left temporal bone
Wiseman et al,6 1995 Middle ear air-fluid level; a 5-mm soft-tissue
structure adjacent to the vertical portion
of the facial nerve
Mulder et al,7 1995 Patient 1: Soft-tissue mass filling the tympanic
cavity; nonaerated pneumatized mastoid
Patient 2: Soft-tissue mass replacing the
vestibule; ossicles not visualized
Patient 3: Soft-tissue mass in the tympanic
cavity; nonaerated pneumatized mastoid
Janicki et al,8 1996 Diffusely enhancing destructive lesion in the
left mastoid and middle ear extending to the
posterior fossa dura
Lee HM et al,9 2001 Soft-tissue mass in the right maxillary sinus that
extended into the nasal fossa and right ethmoid
sinus; mild enhancement and some thickening
and remodeling of bone with erosion and
sclerosis in the region of the right ethmo-
maxillary plate
Williamson et al,10 2003 Opacification of the middle ear and mastoid
with ossicular erosion and erosion of the
tegmen tympani and bone overlying the
semicircular canal,
Gasparotti et al,11 2003 Erosive mass in the right mastoid extending
to the attic without ossicular erosion;
dehiscent tegmen tympani; nonaerated
pneumatized mastoid
Cho AH et al,12 2004 Not reported
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MRI
Low-density mass about the right geniculate
ganglion
Homogeneously enhancing soft-tissue mass
in the left mastoid extending into the middle
cranial fossa and petrous apex
Homogeneous enhancement with
isointensity on T1-weighted imaging
Fluid in the mastoid air cells
Enhancing mass in the middle ear, inner ear,
and internal auditory canal
Enhancing mass in the mastoid cavity and
inner ear extending to the internal auditory
canal and cochlea
Strongly enhancing mass in the middle and
inner ear, cochlea, and internal auditory
canal; loss of T2 signal of the inner ear
Increased T2 signal in the left mastoid soft-
tissue mass
Not reported
Abnormal soft-tissue signal intensity in the
left middle ear mastoid, and external auditory
canal; thickening and enhancement of the
adjacent dura; increased T2 signal
in the left temporal lobe
Low signal intensity on T2-weighted
imaging,intermediate on T1 with contrast
enhancement; dural thickening and
enhancement
Mass lesion in the right mastoid, petrous
bone, and middle ear cavity extending to the
adjacent dura, tentorium, and right cavern-
ous sinus with intense homogeneous
enhancement
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 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE

Table 2. Continued

Author CT MRI

McCall et al,14 2006 Not reported Cavernous sinus thrombosis, extensive

meningeal thickening and enhancement
primarily involving the bilateral cavernous
sinuses, the high posterior clinoid area, and
the distal petrous internal carotid artery
canal

Cho KJ et al,15 2007 Sclerotic mastoid air cells; opacification of the Not reported
middle ear cavity, epitympanum, and mastoid
antrum with erosion of the malleus handle
in both ears

Lee RG et al,16 2007 Lesion of the middle ear along the horizontal Not reported
portion of the facial nerve

Diaz et al,17 2007 Patient 1: Erosion of the petrous bone behind 2-cm gadolinium-enhancing mass posterior
the internal auditory canal and into the vestibule to the internal auditory canal with extension
and semicircular canals into the labyrinth with labyrinthine
enhancement

Patient 2: Scalloped bone erosion along the Gadolinium-enhancing lesion filling the
operculum and posterior petrous face extent of the endolymphatic sac along the
posterior petrous apex and abutting the
labyrinth

Lee JH et al,18 2007 Diffuse soft-tissue lesion in the right middle ear Enhancing and isointense to the gray matter
cavity and mastoid antrum on Tl-weighted imaging, low signal intensity
on T2; mass had extended to the right
petrous apex

CT and MRI
Strasnick and Vaughan,19 Patient 1: Erosive lesion of the right temporal bone extending into the middle cranial fossa
2008
Patient 2: Lesion of the right petrous apex with medial extension to the right sphenoid bone
and anterior extension to the cavernous sinus, foramen rotundum, and pterygopalatine fossa;
lesion extension through the petrous portion of the temporal bone into the mastoid cavity

Santaolalla-Montoya Opacification of the middle ear and mastoid
et al,20 2008 with ossicular erosion and erosion of the
tegmen tympani; temporal bone apex
inflammatory changes and significant lateral
skull base enhancement
Homogeneously enhancing soft-tissue mass
of the right temporal bone with focal bone
erosion and dural thickening into the
posterior cranial fossa; marked extension
into the lateral and medial skull base and
cervical spine

Coulson et al,21 2008 Complete opacification of the middle ear cleft,
attic, and mastoid cells; destruction of the
septae between the mastoid cells, but no
erosive changes of the tegmen tympani,
jugular bulb, or lateral semicircular canal
Abnormally enhancing soft-tissue mass in
the right external auditory canal, middle ear,
attic, and mastoid air cells; diffuse involve-
ment of the temporal and parietal brain
parenchyma with contrast enhancement,
extending through the skull base, into the
infratemporal fossa, and medially to involve
the Meckel cavity, the cavernous sinus, and
the foramen ovale

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 AJIBADE, TANAKA, PAGHDA, MIRANI, JYUNG

Table 2. Continued

Author CT MRI

Allona et al,22 2009 Osteolytic lesion affecting the left petrous apex,
the lateral aspect of the clivus, and the adjacent
carotid and jugular foramina and subtle
enhancement with contrast; subtle signs of
bone regeneration with areas of sclerosis that
were patent at 1 yr of follow-up
Heterogeneous but predominantly high
signal intensity on T2-weighted imaging,
and intermediate signal intensity and
contrast enhancement on T1; magnetic
resonance venography demonstrated
permeability of the transverse left venous
sinus, but the distal part of the sigmoid
sinus and the internal jugular vein were not
visualized because they were thrombosed;
diminution of the size and enhancement of
the lesion with contrast, indicating
spontaneous improvement of the lesion at
1 yr of follow-up

CT and MRI

Garg et al,23 2010 Patient 1: Infiltrating skull base lesion extending into the jugular fossa, clivus, and naso-
pharynx, and encasing the left carotid artery (MRI only)

Patient 2: Infiltrative process of the central and right nasopharynx encasing the right internal
carotid artery

Patient 3: Nasopharyngeal and infratemporal fossa mass encasing the right internal carotid
artery; skull base erosion

Patient 4: Large left infratemporal mass extending into the foramen rotundum and the
maxillary sinus

Ajibade et al,* 2010 Soft-tissue mass filling the right mastoid with Mild homogeneous enhancement of the
focal erosion through the tegmen tympani and right mastoid mass
mastoid cortex

* Present case.

tial damage that can be caused to surrounding nerves.
Inflammatory pseudotumors of the facial nerve have
been surgically resected with subsequent restoration of
facial nerve function; steroid therapy may be indicated
for residual disease.6,10
Steroid therapy has also been successfully used to
treat inflammatory pseudotumors of the temporal bone,
although high-dose steroid therapy is associated with a
number of complications, including avascular necrosis
of the hip, osteoporosis, and immunosuppression.15,24,25
Because our patient’s hip and leg pain might have her-
alded avascular necrosis, we reduced his prednisone
dosage, which might have limited the drug’s efficacy.
It has been reported that as many as 78% of all orbital
inflammatory pseudotumors can be initially treated
successfully with steroid therapy alone, although sub-
sequent recurrence rates have ranged from 25 to 52%,
usually within 12 months after steroid treatment has
been tapered or discontinued.24,26 Likewise, in patients
with inflammatory pseudotumors of the lung, steroid
therapy has been shown to reduce tumor size or induce
complete regression.27 However, the results of steroid
therapy are not as promising in patients with skull
base tumors, as recurrences have been noted following
discontinuation of the regimen.13 Further research may
help to standardize steroid dosing and duration for
more effective treatment of temporal bone and skull
base inflammatory pseudotumors.
Radiotherapy has been used to treat (1) inflammatory
pseudotumors in anatomically inaccessible regions such
as the skull base, (2) recurrent or refractory disease, (3)
tumors that exhibit a poor response to steroid therapy,
and (4) patients who cannot tolerate other treatments,
although it is associated with radiation-induced

E12 ■ www.entjournal.com ENT-Ear, Nose & Throat Journal ■ July 2010

 INFLAMMATORY PSEUDOTUMOR (PLASMA CELL GRANULOMA) OF THE TEMPORAL BONE
neoplasms.10,13,28,29 Our patient’s relatively young age
and otherwise good health supported our decision to
encourage steroid therapy rather than radiotherapy
after the first surgical excision. However, during his
second recurrence, we detected no significant mass,
so we recommended radiotherapy. The responses of
orbital inflammatory pseudotumors to radiotherapy
have been encouraging, with reported remission rates
ranging from 66 to 100%.13 One study of inflammatory
pseudotumors of the temporal bone showed a recur-
rence following a subtotal excision and radiotherapy,
while another study did not.30-32
In conclusion, inflammatory pseudotumor rarely
occurs in the temporal bone. Characteristic clinical and
radiographic findings distinguish this condition from
mastoiditis, chronic otitis media, and cholesteatoma.
An accurate diagnosis depends on a careful review of
CT and MRI findings and histopathologic analysis.
These pseudotumors are locally destructive, and they
have the capacity to erode through bone and invade
soft-tissue structures, so they must be treated aggres-
sively. Total surgical excision should be recommended
as the initial treatment, with adjunctive therapy such
as steroids and radiotherapy reserved for residual,
recurrent, or inaccessible tumors.
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