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Inflammatory Myofibroblastic Tumor—A Case Report
Lindfay Laura Lau, Syed Shoeb Ahmad, Anis Farhad and Shuaibah Abdul Ghani
Ophthalmology Department, Queen Elizabeth Hospital, Kota Kinabalu, Malaysia
Abstract This case report highlights a 4-year old child with recurrent bilateral proptosis. Investigations revealed the presence of inflammatory
myofibroblastic tumors in both eyes. These are rarely reported benign tumors. Our case showed unusual features such as orbital involvement,
bony erosion and response to corticosteroids.
Keywords Granuloma, plasma cell, orbital diseases, orbital neoplasms
Disclosure: Lindfay Laura Lau, Syed Shoeb Ahmad, Anis Farhad, and Shuaibah Abdul Ghani have nothing to declare in relation to this article. No funding was received in the
publication of this article.
Compliance with Ethics: All procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki Declaration of 1975
and subsequent revisions, and informed consent was received from the parent/guardian of the patient involved in this case study.
Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and
reproduction provided the original author(s) and source are given appropriate credit.
Received: November 11, 2015 Accepted: March 7, 2016 Citation: US Ophthalmic Review, 2016;9(1):34–6
Correspondence: Syed Shoeb Ahmad, Ophthalmology Department, Queen Elizabeth Hospital, Kota Kinabalu, 88586, Malaysia. E: firstname.lastname@example.org
Inflammatory myofibroblastic tumors (IMTs) are rarely reported, benign
tumors. They are uncommon in the ocular structures. A four-year-old
girl presented to us with bilateral proptosis. Subsequent radiologic and
histopathologic investigations proved it to be bilateral IMT. This case
report is being presented because of its rarity and some of the unique
features of the condition seen in our patient.
A four-year-old, apparently healthy girl presented to Queen Elizabeth
Hospital, Kota Kinabalu two years ago, with a gradually progressive
proptosis, lagophthalmos, and watering from her right eye for the past
eight months, as noted by her mother. There was no blurring of vision,
diplopia, pain, or redness noted. There was no history of trauma, nor
symptoms suggestive of malignancy, thyroid abnormalities, connective
tissue disease, or infection. She was well and active at home. There was
also no family history of malignancy reported.
Examination of the child revealed an active girl with no features of
dysmorphism. The visual acuities were 6/6 in both eyes. The conjunctivae
in both eyes were mildly injected. The rest of the anterior, as well as
posterior, segments were normal bilaterally. She was noted to have
bilateral non-axial proptosis, which was worse on the right eye. The
right eye was deviated downwards and inwards. Exophthalmometer
measurements at 95 mm were 18 mm on the right eye and 16 mm on
the left eye. No lagophthalmos or exposure keratopathy were present.
Her extraocular movements were normal. There was no relative afferent
pupillary defect noted (Figures 1 and 2).
34 Blood investigations consisting of full blood count, liver function tests,
peripheral blood film, thyroid function, and work-up for sarcoidosis were
all normal. Computerized tomography (CT) scan of the thorax, abdomen,
and pelvis showed cervical lymphadenopathy. An ultrasound abdomen
was also performed, but no metastatic lesions were noted.
Magnetic resonance imaging (MRI) of the brain and orbit revealed an
extraconal lesion in the right supero-lateral aspect of the orbit measuring
1.2 x 1.36 x 3.4 cm. The lesion was isotense with grey matter in T1 and
T2 weighted images. There was a homogenous enhancement post
contrast injection. The mass was extending to the orbital apex, causing
widening of the inferior orbital fissure. Erosion of the greater wing of
sphenoid on the same side was noted. The left extraconal lesion measured
1.1 x 1.1 x 4 cm. There was extension of the mass into the apex of the
orbit and the anterior aspect of the cavernous sinus. Minimal erosion of
the greater wing of sphenoid bone was noted. However, no abnormal
enhancements of the optic nerves were seen. There was enhancing
of the dura along the superior sagittal sinus, which showed a normal
signal void. The choroid plexuses on both sides were also enhancing on
contrast injection. Based on the MRI report, the differential diagnoses were:
granulocytic sarcoma, lymphoma, or neuroblastoma (Figure 3).
Subsequently, bone marrow aspirations (BMA) and lumbar puncture
(LP) were performed by the pediatric hematology oncology team. BMA/
immunophenotyping were not suggestive of leukemia. Cerebrospinal
fluid cytology also did not show any malignant cells. A CT scan revealed
cervical lymphadenopathy, but no metastasis in the lungs or bones.
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