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Ocular Oncology 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: syedshoebahmad@yahoo.com 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. Case Report 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. TOUCH ME D ICA L ME D IA