Benign melanocytic tumor in infancy: Discussion on a rare case and review of the literature

Meningeal melanocytoma is an infrequent neoplasm of the central nervous sys tem (CNS), especially in childhood and infancy. It was first described as a n entity different from pigmented meningiomas and schwannomas in 1972, and few cases have been published so far, in this article, a 5-month-old male p atient with meningeal melanocytoma is presented. This midline lesion was lo calized in the posterior fossa and manifested by hydrocephalus. The entire dural origin and extradural growing pattern in addition to the destruction of the adjacent occipital bone were the unexpected presentations since thes e tumors usually tend to locate on leptomeninges and to extend into the adj acent neural compartment rather than the outside. On the other hand, this c ase is the only one which had identical lesions in both surrenal glands and the left renal capsule, the structures containing neural-crest-derived cel ls outside the CNS. The prognostic criteria, differential diagnosis and its embryological aspects are discussed with an extensive review of the relate d existing literature. Copyright (C) 2000 S. Karger AG, Basel.