Pigmented Epithelioid Melanocytoma of the Eyelid: A Case Report

Pigmented epithelioid melanocytoma is a rare, intermediate-grade melanocytic tumor presenting in a broad age range, often as a pigmented skin lesion. It is characterized by heavily pigmented epithelioid and dendritic melanocytes and shares histological features with epithelioid blue nevus and animal-type melanoma. Pigmented epithelioid melanocytoma typically presents as a slow-growing pigmented lesion and may metastasize to regional lymph nodes, although systemic spread is exceedingly rare. The authors report a unique case of pigmented epithelioid melanocytoma in an 11-year-old boy with an asymptomatic, progressively enlarging pigmented lesion on the lower eyelid. A complete excision of the lesion was performed, with the histopathological analysis showing a symmetrical, intensely pigmented, well-circumscribed melanocytic proliferation with no overt malignant features and no mitotic activity. Immunohistochemical analysis demonstrated negative B-type Raf kinase VE1 staining and partial loss of protein kinase A regulatory subunit 1 alpha staining, consistent with pigmented epithelioid melanocytoma. Current literature emphasizes case-by-case management, with complete surgical excision and vigilant follow-up remaining the mainstay of management.