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¹Haydarpaşa Numune Hastanesi, Patoloji Bölümü, İstanbul
²Haydarpaşa Numune Hastanesi, Nöroşirürji Kliniği, İstanbul
³Haydarpaşa Numune Hastanesi, Dermatoloji Kliniği ve I. Radyoloji Bölümü, İstanbul Neurocutaneous melanosis is a rare congenital syndrome characterized by large or multiple congenital pigmented nevi and benign and/ or malignant melanocytic tumors of leptomeninges. The case history and autopsy findings of a 5 year old male with neurocutaneous melanosis was presented in this paper. He had one giant congenital melanocytic naevus and numerous congenital melanocytic nevi with neurological deficitis due to the involvement of spinal cord. Magnetic resonance imaging of the spine demonstrated findings with diffuse intradural mass and leptomeningeal melanosis. The case died in a very short time after rapidly clinical deterioration. The autopsy findings were diffuse malignant melanoma infiltrating the leptomeninges in spinal cord, tumor infiltration in cerebral cortex, cerebellar subarachnoidal and Virchow-Robin spaces, and, diffuse necrotic areas with infiltrated melanotic cells in cerebellum and pons. The individuals with large or multiple congenital nevi, remembering this syndrome, should be closely observed and should be examined for the presence of leptomeningeal melanosis with MRI. Anahtar Kelimeler : Congenital melanocytic naevus, leptomeningeal melanosis, meningeal malignant melanoma, neurocutaneous melanosis