Lin Chin-Ta, Huang Tsai-Wang, Nieh Shin, Lee Shih-Chun
Department of Surgery, Tri-Service General Hospital, National Defense medical Center, Taipei, Taiwan.
Onkologie. 2009 Sep;32(8-9):503-5. doi: 10.1159/000226591. Epub 2009 Jul 24.
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%.
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.
Although MPNST is an aggressive carcinoma with poor survival, aggressive surgical intervention and radiotherapy resulted in good survival.
恶性外周神经鞘瘤（MPNST）被定义为任何起源于或分化为外周神经鞘细胞的恶性肿瘤，具有局部复发和远处转移的高风险。尽管采取了积极的手术和辅助治疗，但MPNST的预后较差，据报道5年生存率约为50%。
我们报告一例颈部MPNST患者，在无病间隔14年后出现多处胸部转移。由于局部复发且肿瘤侵犯胸壁和心包，该患者在过去3年共接受了4次胸部手术。积极的手术干预和辅助放疗取得了良好的效果和长期生存。
尽管MPNST是一种侵袭性强、生存率低的癌症，但积极的手术干预和放疗仍能带来良好的生存效果。
