Neurocutaneous Melanosis with Malignant Leptomeningeal Melanoma

Abstract

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi with either benign or malignant pigmented cell tumors of the leptomeninges. We report a case of NCM in a patient with previously robust health. No neurological deficit was noted in the patient. At the age of 21 years old, blurred vision initially appeared. We were consulted to evaluate cutaneous lesions in the patient. We found an unduly large, hairy, congenital melanocytic nevus predominantly over the patient's torso with multiple variously sized scattered hairy nevi over his trunk. The patient was diagnosed as NCM with malignant leptomeningeal melanoma following pathologic evaluation of the specimen taken druing frontal craniotomy for removal of tumor and decompression. No other malignant change of the cutaneous lesions and internal organs was noted except in the meninges. The patient received treatment with radiotherapy. However, he died within 3 months.