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目的总结颅内非典型畸胎瘤样-横纹肌样瘤(atypical teratoid-rhabdoid,AT-RT)的诊治经验。方法回顾性分析3例诊断为颅内AT-RT病人的临床资料,其中儿童2例,成人1例,总结其临床特点、神经影像学特点、手术治疗及预后。结果肿瘤全切除2例,近全切1例。术后放疗2例,随访6～13个月,3例病人目前均存活。结论颅内AT-RT是罕见的高度恶性肿瘤,总体预后较差,手术是本病主要的治疗手段。
Objective To summarize the diagnosis and treatment of atypical teratoid-rhabdoid (AT-RT). Methods The clinical data of 3 patients diagnosed as intracranial AT-RT were retrospectively analyzed. Among them, 2 were children and 1 was adult. The clinical features, neuroimaging features, surgical treatment and prognosis were summarized. Results Total resection in 2 cases, nearly complete resection in 1 case. Postoperative radiotherapy in 2 cases, followed up for 6 to 13 months, 3 patients are currently alive. Conclusion Intracranial AT-RT is a rare and highly malignant tumor with poor overall prognosis. Surgery is the main treatment for this disease.