Clinical Report: Rare Sporadic Intramedullary Malignant Peripheral Nerve Sheath Tumor

Background

Intramedullary MPNSTs are exceptionally rare, particularly in sporadic cases without neurofibromatosis type 1 (NF−1) or prior radiotherapy. These tumors are aggressive and associated with high recurrence and metastatic potential.

Data Highlights

No numerical or trial data presented in the article.

Key Findings

• A 22-year-old male presented with progressive left-sided weakness and gait imbalance.
• Imaging revealed an intramedullary C2 mass with intracranial extension and a dorsal subpial nodule at C5.
• Gross total resection confirmed the diagnosis of MPNST.
• Recurrence occurred 18 months later with left cerebellar extension and immunophenotypic dedifferentiation.
• The patient died 14 months after recurrence, indicating the aggressive nature of sporadic intramedullary MPNST.

Clinical Implications

Early recognition of atypical imaging features suggestive of dissemination is important for timely intervention. Multimodal treatment strategies may be necessary to manage the aggressive behavior of these tumors.

Conclusion

This case underscores the need for heightened awareness of sporadic intramedullary MPNSTs and their potential for rapid progression despite surgical intervention.

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