Primary melanocytic tumors of the CNS

Primary melanocytic tumors of the central nervous system are a group of related neoplasms of variable aggressiveness which are thought to arise from leptomeningeal melanocytes which are of neural crest origin.  Under the current (2016) WHO classification of CNS tumors, four entities are recognized :

• meningeal melanocytosis
• meningeal melanomatosis
• meningeal melanocytoma
• meningeal melanoma

Primary melanocytic tumors should not be confused with melanocytic variants of other tumors (e.g. melanotic meningioma, melanotic schwannoma). Occasional melanization is also encountered in gliomas, medulloblastomas and paragangliomas .

All these entities are rare and are far less common than metastatic malignant melanoma.