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[目的]探讨影响恶性周围神经路瘤的诊治和预后因素。[方法]回顾性分析22例头颈部恶性周围神经鞘瘤的临床表现及治疗结果，共复习有关文献。[结果]头颈部恶性周围神经鞘瘤的预后较差，总的5年生存率为26．3％。肿瘤＞5cm者及伴有神经纤维瘤病Ⅰ型者预后较差。扩大手术范围能改善预后。[结论]头颈部恶性周围神经鞘瘤是一种较少见治疗较困难的恶性肿瘤。早期发现，包括对神经纤维瘤病Ⅰ型患者密切随访是提高生存率的关键。
[Objective] To discuss the diagnosis, treatment and prognostic factors of malignant peripheral nerve tumors. [Methods] The clinical manifestations and treatment results of 22 cases of malignant peripheral nerve sheath tumors of head and neck were retrospectively analyzed. [Results] The prognosis of malignant peripheral nerve sheath tumors in head and neck was poor, with a total 5-year survival rate of 26.3%. Patients with tumor >5cm and neurofibromatosis type I have poor prognosis. Expanding the range of surgery can improve the prognosis. [Conclusion] Malignant peripheral nerve sheath tumors of head and neck are rare malignant tumors. Early detection, including close follow-up of neurofibromatosis type I patients, is the key to improving survival.