中枢神经系统胚胎性肿瘤非特指型临床特点和治疗体会

Clinical features and treatment of central nervous system embryonal tumor, not otherwise specified

目的总结中枢神经系统(central nervous system,CNS)胚胎性肿瘤非特指(not otherwise specified,NOS)型患者的临床资料,并对其临床特点和诊疗进行总结和讨论。方法回顾2014年1月—2016年12月间四川大学华西医院神经外科颅内肿瘤患者中,符合CNS胚胎性肿瘤NOS型诊断标准的7例患者,对其临床资料、诊治经过、临床特点进行回顾性分析。结果 7例患者中男4例,女3例;平均年龄25.4岁;病变位于大脑半球5例,位于第三脑室2例。临床症状主要为高颅压引起的头痛、恶心、呕吐,伴有局灶性神经功能障碍。患者均于全身麻醉下行开颅手术切除病变。术后行病理诊断确诊为CNS胚胎性肿瘤NOS型。术后随访6个月~3年,2例死亡。结论 CNS胚胎性肿瘤NOS型是颅内恶性程度较高的肿瘤,该病已被列为CNS胚胎性肿瘤的一大亚类。术前MRI表现具有一定特征性,通过规范化的外科为主的综合治疗,患者能有效延长生命,部分患者可获得较长生存期和较高生活质量。

Objective To review the clinical records of patients with central nervous system（CNS） embryonal tumors, not otherwise specified（NOS）; and summarize their clinical features, diagnosis, and treatment. Method We reviewed the data of patients with intracranial tumors admitted to Department of Neurosurgery of West China Hospital,Sichuan University from January 2014 to December 2016, and retrospectively analyzed the clinical features, diagnosis, and treatment of seven patients with CNS embryonal tumors, NOS. Results There were 4 males and 3 females, and the mean age was 25.4 years old. The tumor was located in cerebral hemisphere in 5 patients, and in third ventricle in 2. Clinical presentation included headache, nausea, and vomiting due to intracranial hypertension, and focal neurological signs. All patients underwent craniotomy for tumor resection and postoperative pathology confirmed CNS embryonal tumor, NOS.The patients were followed up for 6 months to 3 years, and 2 patients died during follow-up. Conclusions CNS embryonal tumor, NOS is malignant intracranial lesion, and has been enlisted as a separate entity under classification of CNS embryonal tumors. It has its unique radiological features, including rare occurrence of perilesional edema, cystic changes, and clear demarcation. Through comprehensive treatment including surgical resection, chemotherapy, and radiation therapy, patients can enjoy prolonged survival and improved quality of life.