儿童颅内非典型畸胎样/横纹肌样瘤1例  

A typical case of atypical teratoid/rhabdoid tumor

作　　者：尹国阳[1] 司俊臣 肖以磊[1] 许崇福[1] 高志宇[1] 蒋海涛 冉然 杨凤海[1] 傅强[1] YIN Guoyang;SI Junchen;XIAO Yilei;XU Chongfu;GAO Zhiyu;JIANG Haitao;RAN Ran;YANG Fenghai;FU Qiang(Department of Neurosurgery,Liaocheng People's Hospital,Liaocheng 252000)

机构地区：[1]山东省聊城市人民医院脑科医院神经外科,聊城252000

出　　处：《中国神经精神疾病杂志》2022年第2期112-115,共4页Chinese Journal of Nervous and Mental Diseases

基　　金：泰山学者工程(编号:tsqn202103200);山东省中医药科技面上项目(编号:2021M033)。

摘　　要：报告1例罕见的非典型畸胎样/横纹肌样瘤。患儿男,5岁,于2017年7月17日因“头痛伴恶心、呕吐2个月,视力减退2 d”入院。查体见:双眼视力约0.1,余未见明显异常。术前磁共振示右额颞叶占位性病变。入院后给予补液、脱水降颅压、抑酸等对症治疗,于2017年7月20日行开颅肿瘤切除术,术中快速病理示:小圆细胞恶性肿瘤。术后病理结果:颅内小圆细胞恶性肿瘤,结合免疫组化结果符合非典型性畸胎瘤样/横纹肌样瘤。术后复查磁共振示肿瘤无残留,患者分别于2017年9月26日、2017年10月17日行2周期化疗,化疗结束后,给予全脑全脊髓放疗。放疗结束后继续给予原方案化疗4周期。随访至2022年1月,多次复查颅脑磁共振未见肿瘤复发,现生活简单自理,就读于一家特殊学校。这提示此类患儿若行肿瘤完整全切除,配合早期及时规范的放疗及化疗,或可能获得较为理想的预后。In this case report,we described a rare case of atypical teratoid/rhabdoid tumor.A 5-year-old male patient was admitted to the hospital on July 17,2017 due to"headache accompanied by nausea and vomiting for 2 months and vision loss for 2 days".Physical examination showed that the visual acuity of both eyes was about 0.1,and there were no other obvious abnormalities.Preoperative MRI Revealed a space-occupying lesion in the right frontotemporal lobe.After admission,the patient was given symptomatic treatment such as fluid rehydration,dehydration to reduce cranial pressure and acid inhibition.Craniotomy of tumor was performed on July 20,2017.Rapid pathology during the operation revealed small round cell malignant tumor.Postoperative pathological results showed that intracranial small round cell malignant tumor,combined with immunohistochemical results it consistent with atypical teratoma/rhabdomyoid tumor.Postoperative magnetic resonance imaging showed no tumor residue.The patients received 2 cycles of chemotherapy on September 26,2017 and October 17,2017,respectively.After the chemotherapy,the whole brain and spinal cord radiotherapy was given.After radiotherapy,the original chemotherapy regimen was continued for 4 cycles.He was followed up until January 2022.Repeated brain MRI examination showed no tumor recurrence.Now he is living a simple life and studying in a special school.This study suggests that if such children receive complete and total tumor resection,combined with early and timely standardized radiotherapy and chemotherapy,they may have a relatively favorable prognosis.

关 键 词：非典型畸胎样/横纹肌样瘤 儿童 胚胎性肿瘤 综合治疗 放疗 化疗 开颅术 

分 类 号：R651[医药卫生—外科学]