A large sporadic melanotic schwannoma was found at the C2-C3 level in a 66-year-old woman who presented a pyramidal syndrome of all four limbs. The diagnosis was established by histology and immunohistochemical studies and confirmed by ultrastructural examination. Thirty-six cases of melanotic schwannoma located in the spinal cord have been reported in the literature, allowing an analysis of the different histological, clinical, and therapeutic aspects. Usually considered as benign tumor, melanotic schwannoma can recur and progress to malignancy, requiring long-term follow-up, specially in patients with multiple tumors and/or Carney's syndrome.
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