Primary melanocytic tumours 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 tumours, four entities are recognised 1:
Primary melanocytic tumours should not be confused with melanocytic variants of other tumours (e.g. melanotic meningioma, melanotic schwannoma). Occasional melanization is also encountered in gliomas, medulloblastomas and paragangliomas 1.
All these entities are rare and are far less common than metastatic malignant melanoma.