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Neurothekeomas (NTK) are benign soft tissue tumors most commonly occurring in the head, neck and upper extremities of young adults, with a female preponderance. Herein we report 2 cases of ocular NTK presenting as an orbital mass or chalazion. Both cases underwent excision of the mass with histopathologic diagnosis of cellular NTK. Tumor cells for both cases were immunopositive for smooth muscle actin (SMA), microphthalmia-associated transcription factor (MITF) and cluster of differentiation 10 (CD10). S100, human melanoma black 45 (HMB45) and melanoma antigen recognized by t-cell (Melan A) were negative. Both were associated with scattered histiocytes which were positive for cluster of differentiation 68 (CD68). To date, including our 2 cases, there have been 22 cases of ocular and periorbital adnexal NTK reported in the English literature. Although rare, clinicians should include neurothekeoma in their differential diagnosis in patients presenting with a periocular soft tissue tumor.
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Name: Survey of ophthalmology
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