Case Report: Primary sporadic intramedullary malignant peripheral nerve sheath tumor with intracranial extension and a subpial nodule suggestive of early dissemination

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Intramedullary malignant peripheral nerve sheath tumors (MPNSTs) are rare, especially in sporadic cases without neurofibromatosis type 1 or prior radiotherapy.
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A 22-year-old male presented with progressive left-sided weakness and MRI revealed an intramedullary C2 mass with intracranial extension.
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Gross total resection confirmed MPNST, but recurrence occurred 18 months later with left cerebellar extension and immunophenotypic dedifferentiation.
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The patient experienced rapid clinical deterioration and died 14 months after recurrence, highlighting the aggressive nature of sporadic intramedullary MPNST.
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Early recognition of atypical imaging features and consideration of multimodal treatment strategies may be important for managing sporadic intramedullary MPNST.

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