非典型畸胎样／横纹肌样瘤七例报告并文献复习

Atypical teratoid/rhabdoid tumor ： 7 cases report and review of literatures

目的探讨非典型畸胎样／横纹肌样瘤的流行病学特点、发病机制、治疗以及预后。方法回顾性分析2007年9月至2008年9月于首都医科大学附属天坛医院神经外科手术治疗的7例患者临床、影像和病理资料以及术后随访情况。结果本组7例均手术切除肿瘤，1例术后放疗，2例术后放疗和化疗。6例患者死亡，术后平均生存期不超过4个月。存活的1例患者术后10个月肿瘤已复发。结论非典型畸胎样／横纹肌样瘤是颅内罕见的高度恶性肿瘤，尽管采取了综合治疗措施，患者预后极差。

Objective To discuss the epidemiology and clinical features of atypical teratoid/ rhabdoid tumor（AT/RT） and to explore its pathogenesis, treatment, and prognosis. Method A retrospective review was conducted in 7 patients with surgically and pathologically verified AT/RT. Results Total or subtotal removal was achieved in 7 cases. 1 case received radiotherapy and 2 cases received both radiotherapy and chemotherapy. Six patients died with mean survival time of less than 4 months after operation. One patient suffered from tumor recurrence at the follow - up of 10 months after operation. Conclusions AT/RT is rare and highly malignant in central nervous system. Its prognosis is bad, although comprehensive therapy is achieved.