Neurothekeoma of the eye, conjunctiva and periorbital adnexa. Report of two cases and brief review

Abstract Neurothekeomas (NTKs) 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, microphthalmia-associated transcription factor, and cluster of differentiation 10. S100, human melanoma black 45, 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. To date, including our 2 cases, there have been 22 cases of ocular and periorbital adnexal NTK reported in the English-language literature. Although rare, clinicians should include NTK in their differential diagnosis in patients presenting with a periocular soft tissue tumor.