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Amanda B. Salter, Yoash Enzer, Bassam Aswad, Michael Chaump; Soft Tissue Perineurioma Of The Lacrimal Gland: A Case Report. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6754.
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Perineuriomas are uncommon benign peripheral nerve sheath tumors composed entirely of perineurial cells. We present a novel case of a soft tissue perineurioma of the lacrimal gland.
A 17-year-old Caucasian male presented with an enlarging, painless, mass in his left upper eyelid. A CT scan demonstrated a well-circumscribed lacrimal gland mass without bony erosion. Based on initial imaging the presumed diagnosis was a pleomorphic adenoma of the lacrimal gland, and a complete excision of the lacrimal gland was performed. A 1-cm lacrimal gland mass was excised. The tumor was well circumscribed, nonencapsulated and composed of fascicular, whorled, and focally storiform spindle cells, with variable cellularity and low mitotic activity. Immunohistochemical analysis demonstrated EMA (epithelial membrane antigen), Claudin-1, CD34 and focal S-100 positivity, and negative staining for keratin, CD31, desmin, and smooth muscle antigen. The morphology and immunohistochemical findings were supportive of a benign soft tissue perineurioma. At three month follow up the patient was doing well, without signs of complication or recurrence.
Perineuriomas are rare neoplasms of the peripheral nerve sheath. Histological subtypes include extraneural (soft tissue) and intraneural groups. Extraneural tumors generally arise in the superficial soft tissues of the extremities and trunk. To our knowledge this is the first report of a soft tissue perineurioma involving the lacrimal gland.
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