Objective:

To present a rare case of sporadic intramedullary malignant peripheral nerve sheath tumor (MPNST) and its aggressive clinical course, highlighting its occurrence without neurofibromatosis type 1 (NF1) or prior radiotherapy.

Approach:

• Case Presentation: A 22-year-old male presented with a four-month history of progressive left-sided weakness, gait imbalance, and sensory disturbance. MRI revealed an intramedullary tumor at C2 with intracranial extension and a small dorsal subpial enhancing nodule at C5, raising concern for early neuraxial dissemination. Gross total resection was achieved.
• Recurrence: Eighteen months post-surgery, the patient experienced recurrence with left cerebellar extension and rapid clinical deterioration.

Key Findings:

• Intramedullary MPNSTs are extremely rare, especially in sporadic cases without NF1 or prior radiotherapy.
• The patient exhibited aggressive tumor behavior with rapid progression and recurrence despite gross total resection.
• Imaging features suggested early dissemination, which is atypical for MPNST and may indicate a more aggressive clinical course.

Interpretation:

The case highlights the aggressive nature of sporadic intramedullary MPNST and the significance of atypical imaging features.

Limitations:

• Limited number of reported cases makes it difficult to generalize findings.
• The patient's decision to decline adjuvant radiotherapy may have influenced the clinical outcome.

Conclusion:

Sporadic intramedullary MPNSTs can exhibit rapid progression and recurrence.