Primary leptomeningeal melanoma in association with neurocutaneous melanosis: A case report

Abstract

Background: Primary melanocytic tumors of the central nervous system accounts for approximately 1% of all melanoma with a peak incidence in the fourth decade. The tumor originates from leptomeningeal melanocytes with a variable degree of belligerence. The proliferation of these melanocytes in large amounts in the dermis and nervous system can raise suspicion of neurocutaneous melanosis (NCM), which is an association between malignant melanoma and the presence of a giant intradermal nevus. Case Description: We present a case of a 62-year-old South Asian male with a large congenital melanocytic nevus (>20 cm in size) in the left hemifacial, and head region who presented with complaints of a single episode of grand-mal seizure followed by neuropsychiatric symptoms. The patient was thoroughly evaluated both clinically and surgically leading to a rare diagnosis of primary leptomeningeal melanoma of the left temporal lobe. The patient subsequently underwent a neuronavigation guided left temporal craniotomy with gross total resection of the lesion. Conclusion: Primary leptomeningeal melanoma with a clinical association with NCM is rarely ever reported within the literature. To date, our case is one of the very few instances where such an association is being reported in this age group along with rare neuropsychiatric symptoms.