Background: Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor deriving from or differentiating into cells of the peripheral nerve sheaths, with a high risk of local recurrence and distant metastasis. Despite aggressive surgery and adjuvant therapy, the prognosis of MPNST is poor with reported 5-year survival rates of about 50%. Case Report: We report a case of a patient with MPNST of the neck, who developed multiple thoracic metastases after a 14-year disease-free interval. The patient underwent a total of 4 thoracic surgeries in the last 3 years because of local recurrence with tumor invasion to the chest wall and pericardium. Aggressive surgical intervention and adjuvant radiotherapy resulted in good outcome and long-term survival. Conclusion: Although MPNST is an aggressive carcinoma with poor survival, aggressive surgical intervention and radiotherapy resulted in good survival.

Keywords:
Malignant peripheral nerve sheath tumor, MPNST, Malignant schwannoma
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© 2009 S. Karger AG, Basel
2009
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