儿童胰母细胞瘤9例病例报告并文献复习  

Pancreatoblastoma in children：9 case reports and literature review

作　　者：李静[1] 张伟令[1] 黄东生[1] 胡慧敏[1] 王一卓[1] 文圆[1] 韩涛[1] 李凡[1] 陈丽萍[1] 

机构地区：[1]首都医科大学附属北京同仁医院儿科,100176

出　　处：《中华实用儿科临床杂志》2017年第15期1162-1165,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：北京市亦庄经济技术开发区领军人才计划[京（2016）2号]

摘　　要：目的总结胰母细胞瘤（PB）患儿的临床特点、治疗情况及预后。方法回顾性分析2008年7月至2016年2月首都医科大学附属北京同仁医院收治的9例PB患儿，男4例，女5例，总结分析其临床特点、治疗及预后特点。结果1．就诊时临床表现：腹部包块6例，上腹或脐周痛2例，皮肤黄染1例；胰腺头部肿瘤5例，胰腺体尾部肿瘤各2例。初诊时4例为胰腺局部肿块；3例为胰腺局部肿瘤伴胰外组织或邻近脏器浸润，1例患儿在治疗过程中出现肝脏转移；2例起病时即有肝脏转移。2．酶学及肿瘤标志物：甲胎蛋白（AFP）升高5例（48．5—52971．0μg／L）；神经元特异性烯醇化酶（NSE）升高5例（22．4—53．4μg／L），糖类抗原125（CA-125）[（40．3—122．8）×10’U／L]升高5例，糖类抗原19-9（CA19-9）升高2例[（65．4～362．5）×10。U／L]，血乳酸脱氢酶（LDH）升高2例（380—838U／L），血淀粉酶升高2例（105．5～122．8U／L）。3．治疗及预后：9例患儿中，直接手术联合化疗6例，其中5例完全缓解，1例停止化疗后9个月复发；化疗后手术2例，其中1例完整切除，术后未治疗，1年后复发；1例部分切除，停止化疗后6个月复发；1例因肿瘤巨大无法手术，化疗不能控制肿瘤进展，未行手术，死亡。上述3例复发患儿目前仍在化疗中。中位随访时间为21个月（8～98个月）。结论AFP增高可作为胰母细胞瘤监测指标之一，手术能否完全切除肿瘤是影响预后的重要因素，辅助性化疗可能会减少复发、延长生存期。Objective To analyze the clinical features, treatment and prognosis of the pancreatoblastoma （PB）. Methods A total of 9 patients including 4 boys and 5 girls diagnosed as pancreatoblastoma in Beijing Tongren Hospital Affiliated to Capital Medical University between July 2008 and February 2016 were enrolled. The clinical fea- tures,therapy and prognosis were analyzed. Results （ 1 ） Clinical manifestations ： 6 cases had abdominal mass, 1 case had jaundice ,2 cases had abdominal pain at the time of diagnosis ;5 cases were found in caput pancreatis ,2 cases were found in corpora pancreatis and 2 cases were found in cauda pancreatis ;4 cases had local tumor,3 cases had local inva- sion, and 2 cases had liver metastasis at diagnosis. （2） Enzymology and tumor marks ： elevated alpha - fetoprotein （AFP） was found in 5 cases （48.5 -52 971.0 μg/L） ,elevated neuron- specific enolase（NSE） was found in 5 cases（22.4- 53.4 μg/L）, elevated carbohydrate antigen 125 （ CA - 125 ） was found in 5 cases [ （dO. 3 - 122.8） x 103 15/ L ], elevated carbohydrate antigen 19 - 9 （ CA19 - 9 ） was found in 2 cases [ （ 65.4 - 362.5 ） x 10^3 U/ L ] , elevated lactate dehydro- genase was found in 2 cases（380 ~ 838 U/L） and elevated hemodiastase was found in 2 cases （ 105.5 - 122.8 U/L）. （3） Treatment and prognosis：in 9 patients,the tumors were resected at first in 6 patients, and 5 of them remitted until now, 1 of them relapsed after the comprehensive treatment for 9 months. Two of the patients underwent operation after the chemotherapy, in which 1 case received complete resection but without chemotherapy after the surgery, whom re- lapsed 1 year later; and the other one was resected partly. But after stopping the comprehensive treatment for 6 months, the disease relapsed. One patient could not receive operate because of huge tumor size, and the chemotherapy failed to control tumor progression;the patient died without operation. Three relapsed children were still in chemotherapy. The

关 键 词：胰母细胞瘤 治疗 预后 

分 类 号：R735.9[医药卫生—肿瘤]