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CASE REPORTS

PhiliPPine Journal of otolaryngology-head and neck Surgery 31

ABSTRACT

Objective: To present a case of a benign orbito-maxillary tumor behaving as an invasive,
expansive malignancy.

Design: Case report

Setting: A tertiary care hospital in Metro Manila

Patient: A 4 ½-year-old boy with a seven month history of right orbito-maxillary mass, proptosis
and epiphora.

Result: A series of biopsies were done before a definite diagnosis was made due to inconsistencies
in the histopathologic findings, clinical picture, and the radiologic presentation of the case. After
diagnosis, appropriate intervention resulted in a dramatic decrease in the size of the mass. At
present, the patient is disease-free and asymptomatic.

Conclusion: Histopathologic diagnosis of Inflammatory Pseudotumor is difficult and
differentiating it from malignant tumors is often a concern for otolaryngologists and pathologists.
In spite of an initial malignant biopsy result, the combination of clinical signs and symptoms and
radiologic findings of an infiltrative mass lesion, should not discount the possibility of a benign
entity such as Inflammatory Pseudotumor for which treatment is conservative.

Keywords: Orbito-Maxillary Mass, Inflammatory Pseudotumor, Pseudotumor, Orbital
Pseudotumor

AN ORBITO-MAXILLARY mass may primarily be an orbital lesion extending into the
maxillary sinus or a primary maxillary sinus lesion extending into the adjacent orbit. We present
a case of 4 ½-year-old boy with an invasive right orbito-maxillary mass.

CASE REPORT
Seven months prior to consult, our patient presented with right lower eyelid swelling (Figure

1-A and 1-B) accompanied by non-productive cough and watery nasal discharge. There was no
history of trauma. Maternal and birth history were unremarkable. Developmental history was at
par with age. Past medical history included an allergy to contrast (dye) with a family history of
brain cancer (maternal grandmother) and leukemia (paternal uncle). He was brought to an ear,
nose and throat (ENT) specialist who diagnosed sinusitis and gave unrecalled antibiotics for 2
weeks which did not improve his condition.

Six months prior, the persistence of the symptoms prompted a consultation with another ENT

specialist. Orbital magnetic resonance imaging (MRI) revealed a 3.2 x 2.7 x 2.8 cm mass within the
inferior half of the right orbit and the right maxillary sinus with destruction of the orbital floor
(Figure 2). There was also superior displacement and proptosis of the right globe.

He was referred to an ophthalmologist who biopsied the mass via an infraciliary approach.
The report revealed “small round cell tumor to consider non-Hodgkin’s lymphoma,
Neuroendocrine tumor “(Figure 3) with a note from the pathologist that the specimen had

Inflammatory Pseudotumor of an Orbito-
Maxillary Mass Masquerading as a MalignancyMaria Cristina C. da Silva, MD1,

Joel A. Romualdez, MD1,
Norberto V. Martinez, MD 1,2

1Department of Otolaryngology Head and Neck Surgery
St. Luke’s Medical Center
2 Department of Otolaryngology Head and Neck Surgery
Santo Tomas University Hospital

Correspondence: Maria Cristina C. da Silva, MD
Department of Otolaryngology Head and Neck Surgery
St. Luke’s Medical Center
279 E. Rodriquez Ave, Quezon City 1102
Philippines
Telefax: (632) 727 5543
E-mail: peachiemd@yahoo.com
Reprints will not be available from the author.

No funding support was received for this study. The authors
signed a disclosure that they have no proprietary or financial
interest with any organization that may have a direct interest
in the subject matter of this manuscript, or in any product
used or cited in this report.

Presented at the Interesting Case Contest (2nd Place)
Philippine Society of Otolaryngology – Head and Neck
Surgery Midyear Convention, Puerto Princesa City, Palawan,
April 2005. Philipp J Otolaryngol Head Neck Surg 2006; 21 (1,2): 31-36 c Philippine Society of Otolaryngology – Head and Neck Surgery, Inc.

Silver anniverSary iSSue vol. 21 noS. 1 & 2 January –June; July – december 2006

CASE REPORTS

32 PhiliPPine Journal of otolaryngology-head and neck Surgery

FIGURE 1-A: Right lower eyelid
swelling

FIGURE 3: Small round cell tumor to consider Non-
Hodgkin’s Lymphoma, Neuroendocrine tumor

excessive crushing artifact precluding a definite diagnosis. A repeat
biopsy was suggested.

A trial of oral Prednisone at 0.2 mg/kg/day or 4 mg daily for 1
month apparently decreased the size of the mass. The dose was then
tapered to 2 mg once a day then 1 mg daily for 2 weeks each but the
mass increased in size again. After 2 months of steroid therapy, an
orbital computed tomography (CT) scan revealed a 3.6 x 2.7 x 2.7 cm
mass within the right orbit and maxillary sinus (Figure 4) slightly larger
than previously seen on MRI. Orbital floor lysis, as well as the superior
displacement and proptosis of the right globe, were still evident.

The patient was referred to our institution for further management.
He presented with a 2 x 2 cm mass over the right lower eyelid and slight
proptosis of the right eyeball with unremarkable visual acuity, extra-
ocular muscle (EOM) motility, and fundoscopic exam. An incision biopsy
via Caldwell-luc approach revealed “small round cell tumor, consider
olfactory neuroblastoma” (Figure 5).

Special staining was negative for Chromogranin and LCA (leukocyte
common antigen) but NSE (neuron-specific enolase) was positive,
consistent with olfactory neuroblastoma. A positive NSE does not
confirm the diagnosis nor does a negative chromogranin and LCA
rule it out because it is not unusual for this kind of tumor to stain only
focally. A repeat biopsy was again recommended by the pathologist.
We requested a CT scan of the paranasal sinuses (Figure 6), which did
not show interval changes from previous CT and MRI studies.

Closer examination of all radiologic studies revealed an
unremarkable olfactory cleft with no sign of sinus wall bowing
characteristic of an olfactory neuroblastoma. Clinically, the mass
had not increased in size though 6 months had passed since the first
biopsy. Doubting the diagnosis of olfactory neuroblastoma for lack of
clinical and radiologic corroboration, another biopsy was done. The
histopathologic reading of “chronic and acute inflammation negative
for malignancy” (Figure 7) was not compatible with the bony lysis seen
on CT and MRI.

We decided to debulk the tumor for both diagnostic and

therapeutic purposes. The specimen was signed out as “Inflammatory
Pseudotumor, right infraorbital area” (Figure 8).

The patient was given oral Prednisone at 2 mg/kg /day or 40 mg

daily for 2 weeks, 32 mg once a day for 2 weeks, 24 mg once a day
for 2 weeks and 24 mg daily for 4 weeks. 5 months post-surgery, the
patient remained asymptomatic with no sign of recurrence of the mass
(Figure 9). A repeat CT scan of the paranasal sinus revealed significant
regression of the previously noted inferior orbital soft tissue mass (1 x
0.4 cm) (Figure 10-A). The proptosis and superior displacement of the
right globe was no longer appreciated (Figure 10-B). Steroid therapy
was discontinued and a 6-month review scheduled.

FIGURE 1-B: View from the right
side

R L

FIGURE 2: Orbital MRI: Mass within the inferior half of
the right orbit and right maxillary sinus

R L

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DISCUSSION
Orbital mass lesions may arise primarily within the orbit, extend

from contiguous structures, or be metastatic from a distant primary
malignancy. Primary orbital masses include congenital, vascular, neural,
and mesenchymal tumors. Other types of lesions masquerading as
true neoplasms include lymphoproliferative disorders, autoimmune
diseases, and infectious processes. Congenital lesions include dermoid
cysts, hamartomas and teratomas. The following table (Table 1) shows
the most common pediatric tumors which present in the orbital area.

An olfactory neuroblastoma was considered in our second biopsy
but CT and MRI studies of our patient did not support this diagnosis.
Olfactory neuroblastoma (Esthesioneuroblastoma) displays a variety of
imaging characteristics and aggressiveness, characterized by bowing
of the sinus walls, usually replacing the turbinates, septum, and sinus
walls with extension into contiguous areas 12. Not only did CT and MRI
studies of our patient not show any of these findings, the unremarkable
olfactory cleft cast more doubt on this diagnosis.

The acute and chronic inflammation seen on the third biopsy was
possible following initial response to steroids but does not explain the
lysis or bone destruction evident in the CT and MRI studies. The great
discrepancy between malignancy and mere inflammation prompted
tumor debulking to obtain a larger specimen and once and for all,
put this diagnostic dilemma to rest, finally revealing inflammatory
pseudotumor.

The first specimen had excessive crushing artifacts. Microscopically,
small round cells which stained blue were identified. Small round
inflammatory cells seen clumped or grouped together can make them
seem neoplastic. Because small round cells are non-specific, special
stains are needed (Table 2). Unfortunately, the excessive crushing of the
specimen made this virtually impossible to accomplish.

The next biopsy again showed small round blue cells in clusters
suggesting a neoplasm. Special staining negative for Chromogranin
and LCA but positive for NSE supported a neuroendocrine tumor
such as olfactory neuroblastoma but was not confirmatory. NSE is
not a specific marker and needs chromogranin to stain positive for
the diagnosis to be consistent with olfactory neuroblastoma. The
pathologist could not commit to a diagnosis and suggested another
biopsy, which gave a clue to the inflammatory nature of this disease.
Microscopically, inflammatory cells such as lymphocytes, plasma cells
and histiocytes were seen. However, the specimen was signed out only
as acute and chronic inflammation because such cells are non-specific
findings in any inflammatory process. But these cells also could very
well be present at the surface of any malignant tumor. Tumor debulking
provided a clearer pattern of cellular distribution with inflammatory
cells present throughout the whole specimen. The pathological picture
of dense fibrous tissue with aggregates of lymphocytes, plasma cells
and histiocytes is the hallmark of inflammatory pseudotumor, but this
basis is non-specific3. It is precisely the non-specific nature of the lesion
which makes diagnosis difficult and limits the diagnostic yield of small
biopsies. It was not that the specimens submitted were inadequate; the
non-specific nature of the lesion could only be confirmed by getting the
specimen in toto – a practice not commonly employed when dealing

FIGURE 5: Small round cell tumor, consider
olfactory neuroblastoma

R L

FIGURE 6: PNS CT: No significant interval changes

FIGURE 4: Orbital CT: Slightly larger mass within
the right orbit

R L

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CASE REPORTS

34 PhiliPPine Journal of otolaryngology-head and neck Surgery

FIGURE 9: The patient was asymptomatic 6 months
post-surgery

Table 1. Common Orbital tumors in the pediatric age group

AGE OF
PRESENTATION

2-5 yrs. Old

Before 5 yrs old

2-5 yrs. Old

11-20 and
51-50

Older age group

DISEASE

RHABDOMYOSARCOMA

RETINOBLASTOMA

NEUROBLASTOMA
(METASTATIC)

OLFACTORY
NEUROBLASTOMA

HISTIOCYTOSIS
(CHRONIC FORM)

SEX
PREDILECTION

Male predominance

None

Male predominance

No predilection

INCIDENCE

4.5/ million

1 in 18,000

10/ million

Rare

ORBITAL SIGN/SYMPTOM

Unilateral proptosis

Leukokoria or proptosis

Proptosis

Exophthalmos

with a suspicious malignant-looking mass in an inaccessible area in the
head and neck.

Inflammatory pseudotumor (IPT) is a rarely occurring lesion with
no identifiable local or systemic causes. The lesions mimic expansive,
invasive, malignant tumors both clinically and radiologically, hence the
term “pseudotumor”. IPT most commonly involves the lung and orbit. It
has been reported in nearly every site in the body but is most common
in the orbit and rare in the sinuses5. In our case, we believe that the
mass was primarily an orbital lesion which extended into the maxillary
sinus and not the other way around.

The behavior of IPT can be quite unpredictable3. Some resolve
spontaneously and others respond to corticosteroids. In some cases,
tapering steroid doses cause lesions to recur. For lesions not responding
to steroids, subtotal excision with or without radiotherapy may be
required3.

IPT is an idiopathic inflammatory lesion although various stimuli
may cause it to develop such as unrecognized organisms, minor trauma,
smoking, and chronic irritation by cocaine abuse1. The characteristic
feature of IPT lies not in the inciting agent but in its response to a trigger
agent. According to Williams et al4, the underlying mechanism in the
development of IPT is localized derangement in the immune response
after a particular initial insult. Other authors relate IPT to production of
mediators of inflammation which stimulate proliferation of fibroblasts,
extravasation of neutrophils and activation of procoagulant activity of
the vascular endothelium. It also induces production of acute phase
reactants, proteolysis and neurologic disturbances1.

The most frequent symptom is swelling and pain although other
local symptoms depend on the site of involvement.

A very similar case was seen in Japan by Takashi Nakagawa et.al.2
where a 63 year old male presented with left eye lacrimation and
exopthalmos. CT and MRI studies strongly suggested a malignant
tumor of the maxillary sinus. A biopsy was also done which revealed IPT.
Like our case, this patient did not respond to corticosteroids. Because
total excision of the mass could not be achieved, debulking was done
and there have been no observed further changes in the size of the
mass several months after.

Another study done in New York by PM Som et al6 discussed 6 cases
of IPT of the maxillary sinus. This study showed that IPT of the maxillary

FIGURE 7: Chronic and acute inflammation negative for
malignancy

FIGURE 8: Inflammatory pseudotumor

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PhiliPPine Journal of otolaryngology-head and neck Surgery 35

Table 2.

IMMUNOHISTOCHEMICAL STAINS

LCA

Cytokeratin

NSE

Desmin

Vimentin

Chromogranin

Synaptophysin

TUMORS

Lymphoma

Epithelial tumors

Neuroendocrine tumors

Muscle tumors

Mesenchymal tumors

Neuroendocrine tumors

sinus causes bone changes on CT and MRI, findings that mimic a
malignant tumor.

Heathcote and Safneck3 reported a case of a 5-year-old boy who
presented with eyelid swelling and was admitted with a diagnosis of
orbital cellulitis. A CT scan performed the day after admission revealed
a mass in the lateral orbit. Rather than the presumptive diagnosis of
rhabdomyosarcoma, a biopsy revealed sclerosing IPT.

Among the different ancillary procedures, CT scan has proven to be
of value in defining the extension of IPT and its response to treatment.
In the orbit, the most frequent radiologic characteristics are retrobulbar
fatty infiltration, proptosis, EOM enlargement and apical fat edema.
In extraorbital locations in the head and neck, a mass lesion with
sharp enhancement is consistently reported. IPT has an aggressive
appearance and there is usually bone involvement like erosion,
remodeling or sclerosis.

The unifying histologic feature of IPT is the highly variable mixture
of bland-looking spindle cells and inflammatory cells1. The liberation
of cytotoxic proteins from eosinophil granules may promote fibrosis
and may also contribute to the degeneration of the extraocular muscles
in IPT.

Histopathologic diagnosis of IPT is difficult and differential diagnosis
from malignant tumors is often a concern for otolaryngologists and
pathologists. Hence, coordination between these 2 specialties facilitates
handling such difficult cases as this. The role of the ENT specialist in
correlating history, physical examination, ancillary procedures and
histopathology in spite of disparities cannot be overemphasized. In
spite of an initial malignant biopsy result, the combination of clinical
signs and symptoms and radiologic findings of an infiltrative mass
lesion, should not discount the possibility of a benign entity such as IPT
for which treatment is conservative, and unnecessary and potentially
mutilating surgery can be avoided.

FIGURE 10-A: Regression of the globe soft tissue
swelling

R L

FIGURE 10-B: Proptosis of the right was no longer
evident

R L

ACKNOWLEDGEMENTS:
We thank Dr. Anthony Calibo for help with statistical analysis, Dr. Francisco Narciso for technical

assistance and Drs. Gretchen Navarro-Locsin and Bernabe Singson for their scientific advise.

REFERENCES
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