化疗在儿童颅内生殖细胞肿瘤诊治中的应用  

Application of chemotherapy in the diagnosis and treatment of children with intracranial gonioma

作　　者：李禄生[1] 梁平[1] 李映良[1] 翟瑄[1] 周渝冬[1] 于增鹏[1] 邹彬[1] 纪文元[1] 

机构地区：[1]重庆医科大学附属儿童医院神经外科、儿童发育疾病研究教育部重点实验室、儿科学重庆市重点实验室、重庆市儿童发育重大疾病诊治与预防国际科技合作基地,重庆400014

出　　处：《重庆医科大学学报》2015年第1期73-77,共5页Journal of Chongqing Medical University

基　　金：国家临床重点专科建设资助项目

摘　　要：目的：探讨化疗在儿童颅内生殖细胞肿瘤（germ cell tumors,GCTs）的诊断与治疗中的意义和价值。方法：2010年1月至2013年12月我科拟诊GCTs患儿根据肿瘤标志物甲胎蛋白（alpha-fetoprotin,AFP）和β-人绒毛膜促性腺激素（human chorionic gonadotropin beta,β-HCG）检查结果分为生殖细胞瘤和非生殖细胞瘤性生殖细胞瘤（non-germinoma germ cell tumors,NGGCTs）,生殖细胞肿瘤行CE方案（卡铂＋依托铂苷）,诊断性化疗2个疗程后复查根据实体瘤疗效评价标准（response evaluation criteria in solid tumors,RECIST）判断化疗效果,肿瘤部分缓解（partial remission,PR）或完全缓解（complete remission,CR）临床确诊为GCTs后,≥3岁的患者行降低剂量的放疗及后续化疗,〈3岁的患儿继续化疗,待3岁后延期放疗,排除诊断的则行手术,根据病理检查进一步治疗;内胚窦瘤混合型生殖细胞瘤分别采用BCE（博来霉素、卡铂、依托铂苷）和ICE方案（异环磷酰胺、卡铂、依托泊苷）化疗,同时均联合足量全中枢放疗,视情况行二期探查手术。结果：25例患者获得随访,20例拟诊生殖细胞瘤患者诊断性化疗后PR19例（95%）,临床确诊为生殖细胞瘤后,≥3岁组（15例）,经后续放化疗后100%CR,随访生存率100%,平均随访时间2年,〈3岁组（3例）,1例化疗第6个疗程肿瘤发生全脑全脊髓转移,放弃治疗,2例化疗6个疗程后均顺利完成延期放疗,放化疗结束后均CR,目前随访中肿瘤稳定1例,排除生殖细胞瘤诊断,经手术后病检确诊为畸胎瘤,肿瘤全切,目前无事件生存（event free survival,EFS）2年。诊断NGGCTs（5例）,其中卵黄囊瘤（yolk sac tumor,YST）2例,1例化疗4个疗程,放疗期间复发死亡,1例手术完整切除后顺利完成化疗及放疗,目前已EFS 2年;混合性生殖细胞瘤3例,均行化疗＋放疗＋化疗的综合治疗,EFS1~3年。结论：GCTs对CE方案的化疗非常敏感,诊断性化疗是一种有�Objective：To investigate the role of chemotherapy in the diagnosis and treatment of children with intracranial gonioma.Methods：Cases to be diagnosed as intracranial gonioma in the Children’s Hospital of Chongqing Medical University from January2010 to December 2013 were divided into intracranial gonioma and non-intracranial gonioma according to tumor markers alpha-fetoprotin（AFP）and human chorionic gonadotropin beta（β-HCG）test results. Cases in intracranial gonioma group were treated by CE chemotherapy for two courses,and the curative effect including partial remission（PR）or complete remission（CR）was evaluated using solid tumor evaluation criteria-response evaluation criteria in solid tumors（RECIST）. After making a definite diagnosis,patients over 3 years old carried out radiation therapy and follow-up chemotherapy and patients less than 3 years old were administrated with chemotherapy until over the age of 3 years,and then received extended radiotherapy. Non-intracranial gonioma cases were administered with BCE or ICE chemotherapy combined with full dose of central nervous system radiotherapy. Whether carrying out the secondsurgery was decided according to the actual conditions. Results：Twenty-five patients were followed up,19 patients（95%）out of20 suspected patients with intracranial gonioma were PR after receiving chemotherapy. After being diagnosed as intracranial gonioma,15 patients（100%）in over than 3-year-old group were CR after chemotherapy,with 100% survival rate and an averagefollow-up period of 2 years. In less than 3-year-old group,one patients experienced relapse and craniospinal metastasis after six courses of chemotherapy and abandoned the treatment,the other 2 patients were successfully completed deferred radiotherapy after six courses of chemotherapy,both CR until now. During the current follow-up,one patient with stable tumor was diagnosed as teratomas by pathologic examination excluding the diagnosis of intracranial gonioma and underwent resection. There wer

关 键 词：儿童 颅内生殖细胞肿瘤 化疗 诊断和治疗 

分 类 号：R739.41[医药卫生—肿瘤]