Treatment of a Malignant Peripheral Nerve Sheath Tumor

Abstract

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.