Pigmented epithelioid melanocytoid tumor of the ocular adnexa

An 8-year-old girl with a history of microphthalmia in the right eye presented with a left medial upper eyelid mass with a dark blue-green nodule that could be seen through the skin but did not appear to involve the overlying epidermis. A biopsy demonstrated a pigmented epithelioid melanocytoid tumor with rare mitoses arising in association with a congenital nevus and positive margins. Due to the pathological findings, the patient underwent excision of the tumor with 5-mm margins and a sentinel lymph node biopsy. The re-excision of the upper eyelid margins demonstrated residual benign congenital melanocytic nevus, but did not reveal residual melanocytic lesion. The parotid sentinel node biopsy revealed benign and cytologically mature nevus nests in the capsule and septa, but there was no evidence of involvement with the pigmented epithelioid melanocytoid tumor. The patient underwent successful eyelid reconstruction and had no evidence of recurrence or metastasis after 2 years.