Treatment of a malignant peripheral nerve sheath tumor.
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%.


METHODS
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.


CONCLUSIONS
Although MPNST is an aggressive carcinoma with poor survival, aggressive surgical intervention and radiotherapy resulted in good survival.
Lin
Chin-Ta
CT
Department of Surgery, Tri-Service General Hospital, National Defense medical Center, Taipei, Taiwan.
Huang
Tsai-Wang
TW
Nieh
Shin
S
Lee
Shih-Chun
SC
D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000473:pathology / Q000556:secondary* / Q000601:surgery*; D013899:Thoracic Neoplasms / Q000473:pathology / Q000556:secondary* / Q000601:surgery*; D016896:Treatment Outcome