Supratentorial intermediate grade meningeal melanocytoma with intratumoral bleed in the background of neurocutaneous melanosis: Report of an unusual case and review of literature

Primary melanocytic tumours of the central nervous system (CNS) are rare. According to the WHO classification (2007), these tumours include diffuse leptomeningeal melanosis, melanomatosis, melanocytoma, and primary CNS melanoma. Meningeal melanocytoma, most commonly seen in the infratentorial compartment and cervical spinal cord, is a benign primary melanocytic neoplasm. Primary CNS melanoma, on the other hand, represents the malignant end of the spectrum. Intermediate grade melanocytoma is a rare histological subtype of primary meningeal tumours and is characterised by the clinicopathological features between the two extremes. Neurocutaneous melanosis (NCM) is a rare phacomatosis characterised by melanotic lesions on the skin and leptomeninges. Leptomeningeal manifestation in NCM may be observed either in the form of diffuse leptomeningeal melanosis or primary CNS melanoma. Melanocytomas are focal lesions and their association with NCM is extremely rare. In this report, we present an unusual case of NCM accompanied by right frontal intermediate grade melanocytoma with intratumoral bleeding in a 17-year-old boy. A brief literature review is also presented.

Key-words:

Hemorrhage - hydrcephalus - intermediate grade melanocytoma - neurocutaneous melanosis - supratentorial

Publication History

Article published online:
20 September 2022