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NRAS somatic mutations in diffuse tumors in children Terminology Not recommended: melanoma / melanocytoma without specifying the site ICD coding ICD-O ICD-11 2A0Z - primary melanoma of the central nervous system 2A01.0Y & XH8974 - other specified meningiomas & meningeal melanocytosis 2A01.0Y & XH1BP7 - other specified meningiomas & meningeal melanomatosis 2A01.0Y & XH2RY7 - other specified meningiomas & meningeal melanocytoma 2A01.0Y & XH3DN1 - other specified meningiomas & melanoma, meningeal Epidemiology Diffuse melanocytosis Rare, with difficult to establish incidence Strongly associated with neurocutaneous melanosis (NCM), a rare congenital syndrome characterized by giant congenital pigmented skin nevi and neurological symptoms due to diffuse leptomeningeal melanocytic proliferation (Semin Cutan Med Surg 2004;23:138) Diffuse melanomatosis Bimodal age distribution: onset in childhood in the setting of neurocutaneous melanosis or in adulthood in the fourth decade (Brain Pathol 2015;25:209) Patients with NCM can develop meningeal melanomas in childhood (Pediatr Blood Cancer 2024;71:e30859) Meningeal melanocytoma (J Clin Neurosci 2010;17:1227) Incidence of 1/10,000,000 person years Arises at any age, mostly affects adults Meningeal melanoma (J Clin Neurosci 2010;17:1227) Incidence of 0.005/100,000 person years Reported in children and adults, with a peak in the fifth and sixth decades Sites Diffuse meningeal melanocytosis and melanomatosis Can involve intracranial or spinal leptomeninges Highest frequency in cerebellum, brain stem, temporal lobes and spine (J Am Acad Dermatol 1991;24:747) Meningeal melanocytomas Can occur at any site