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Malignant melanotic nerve sheath tumors account for less than 1% of all primary peripheral nerve sheath tumors and most often arise from the spinal nerves or visceral autonomic nerves ( MMNST has historically been regarded as an atypical variant of schwannoma and, due to its pigmentation, has been referred to as melanotic schwannoma ( In some series, over 50% of patients with MMNSTs have evidence of Carney complex, an autosomal dominant, occasionally familial multiple neoplasia syndrome ( The average age at diagnosis of MMNST is 41 years, with a reported range of 1181 years, which corresponds well to our patient (46 years) ( Table 1 summarizes cases identified in PubMed using the search terms MMNST and malignant melanotic nerve sheath tumor that were published after the 2021 WHO CNS tumor classification, in which MMNST was designated a potentially aggressive condition

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