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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 (31)