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The neurocutaneous melanosis is a rare syndrome in which the congenital melanocytic nevi and excessive melanosis are the main features. The syndrome seems to be a morphogenesis error of the embryonic neuroectoderm. A clinical case with necroscopy in a 19 year-old man who had developed malign melanoma in his central nervous system is reported.

Melanose neurocutânea. Relato de caso com melanoma maligno do sistema nervoso central