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Introduction Primary melanocytic tumors of the central nervous system (CNS) are lesions derived from melanocytes and were first described in 1859 by Rudolf Virchow [ Unlike secondary melanomas that affect the CNS, which have a high incidence, primary melanocytic tumors of the CNS are rare, with an incidence of 0.7 cases per 10 million inhabitants per year [ In the latest classification of CNS neoplasms by the World Health Organization (WHO) in 2021, primary CNS melanocytic tumors were categorized into four types (Table 1): primary leptomeningeal melanoma, primary leptomeningeal melanocytoma, primary leptomeningeal melanomatosis, and primary leptomeningeal melanocytosis [ TABLE 1 Primary melanocytic tumors of the CNS - WHO, 2021

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