多学科协作对儿童原发颅内生殖细胞肿瘤52例的临床诊治及随诊  

Diagnosis,treatment and follow-up of 52 cases of primary intracranial germ cell tumors diagnosed by pathology under the multidisciplinary collaboration

作　　者：张乐嘉[1] 赵雪臻 肖娟[1] 朱慧娟[3] 姚勇[4] 连欣[5] 钟定荣[6] ZHANG Lejia;ZHAO Xuezhen;XIAO Juan;ZHU Huijuan;YAO Yong;LIAN Xin;ZHONG Dingrong(Department of Pediatrics;Department of Endocrinology;Department of Neurosurgery;Department of Radiotherapy;Department of Pathology,Peking Union Medical College Hospital,Beijing 100005,China;Department of Pediatrics,Peking Obstetrics and Gynecology Hospital,Beijing 100062,China)

机构地区：[1]北京协和医院儿科,北京100005 [2]北京妇产医院儿科 [3]北京协和医院内分泌科 [4]北京协和医院神经外科 [5]北京协和医院放疗科 [6]北京协和医院病理科

出　　处：《中国小儿血液与肿瘤杂志》2018年第4期178-183,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的总结经病理明确诊断的儿童原发颅内生殖细胞肿瘤的临床及病理特征,并探讨化疗联合低剂量放疗的临床疗效及转归。方法收集我院2002年12月-2015年12月通过内分泌、儿科、病理科、神外科、检验科、病理科多科协作模式确诊的1～18岁原发颅内生殖细胞肿瘤52例为研究对象,对其临床特征进行回顾性分析。对其中化疗联合低剂量放疗的31例患儿评估疗效,并做初步随诊。结果患儿因尿崩症、生长迟缓、闭经、性早熟等症状首诊于内分泌科或儿科。65%(34/52)的患儿在脑外科行神经内镜下活检/切除术,15%(8/52)行立体定向脑活检术。实验室检查发现60%(27/45)患儿血清βHCG水平正常,13%(6/45)脑脊液βHCG正常。病理检查提示最常见的病理类型为生殖细胞瘤,占87%(45/52),其中混合性生殖细胞肿瘤占10%(5/52)。按病理类型分组,预后不良组2年生存率明显低于预后良好组、预后中等组(P<0.01)。化疗+低剂量放疗后,总有效率达92%,全部患儿无智力障碍、严重神经认知障碍等并发症。结论当血清βHCG水平正常,而临床怀疑颅内生殖细胞瘤时,建议经神经内镜微创活检,以早期明确诊断及病理类型。儿童颅内生殖细胞肿瘤的预后与病理类型有关。基于病理类型的预后分类,可更好地对患儿的转归进行分层,以利于治疗方案的确定和预后的评估。化疗+低剂量放疗的方案取得了较好的疗效,并明显改善了颅内生殖细胞肿瘤的患儿生活质量和神经认知功能。多学科合作模式对于生殖细胞肿瘤的诊治更安全有效。Objective To summarize the clinical and pathological features of primary intracranial germ cell tumors in children diagnosed by pathology And investigate the treatment effect and prognosis of chemotherapy combined with low dose radiotherapy. Methods Collected 52 children（1-18 years old） of primary intracranial germ cell tumors diagnosed and treated by endocrinology, pediatrics, pathology,surgery, pathology and pathology from December 2002 to December 2015 in Peking Union Medical College Hospital. The clinical characteristics of the 52 patients were retrospectively analyzed. Evaluate the efficacy of chemotherapy combined with low dose radiotherapy in 31 children, and follow-up them.Results The children with diabetes insipidus, growth retardation, amenorrhea and precocious puberty saw the endocrinologist or pediatrician at the first time. 65%（34/52） of the patients were made by endoscopic biopsy and resection in the department of cerebral surgery, and 15%（8/52） were made stereotactic brain biopsy. The laboratory examination found that 60%（27/45） of the children with normal serum beta HCG levels, and 13%（6/45） of the children’s cerebrospinal fluid beta HCG normal.So these children have to take a pathological biopsy for diagnosis. The most common pathological type was germ cell tumor 87%（45/52）, followed mixed germ cell tumors 10%（5/52）. According to pathological types, the 2 year survival rate of the poor prognosis group was significantly lower than that of good prognosis group and moderate prognosis group（P 〈 0. 01）. After chemotherapy ＋ low dose radiotherapy,the total effective rate was 92%. And 100% of the children didn’t have mental retardation,severe neurocognitive disorders and other complications. Conclusions When the level of serum beta HCG is normal, and clinical suspicion of intracranial germ cell tumor, it is suggested that endoscopic biopsy is necessary for diagnosis and pathological type. The prognosis of intracranial germ cell tumors in children is related to pat

关 键 词：中枢神经系统 生殖细胞肿瘤 肿瘤标志物 儿童 病理 

分 类 号：R739.41[医药卫生—肿瘤]