Objective and importance: Intramedullary melanotic schwannomas are very rare lesions; only four cases have been reported previously. We describe a patient with an intramedullary melanotic schwannoma that had a more aggressive course than those reported in the literature, and we review the theories regarding the cause of these lesions.
Clinical presentation: A 35-year-old man presented with neck stiffness and paraesthesia extending down his right arm upon neck extension. A magnetic resonance imaging scan revealed an intramedullary lesion extending from C4 to C5.
Intervention: Gross total resection of the mass was performed, and pathological characteristics were consistent with a melanotic schwannoma. Two years after resection the tumor recurred, and the patient was treated with radiation therapy. The tumor progressed 2 years after radiotherapy, and at repeat resection, multiple pigmented foci were present on the surface of the spinal cord and dura consistent with metastatic seeding.
Conclusion: In a patient with intramedullary melanotic schwannoma with an unusually aggressive course, careful follow-up may be essential.