肝母细胞瘤发生中枢神经系统转移12例的临床特点、治疗及预后分析  

Clinical features,treatment and prognosis of 12 patients with primary hepatoblastoma combined with centralnervous system metastasis

作　　者：张伟令[1] 胡慧敏[1] 黄东生[1] 李静[1] 文圆[1] 韩涛[1] 李凡[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1158-1161,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：北京同仁医院院内基金（2015-YJJ-GGL-009）

摘　　要：目的探讨肝母细胞瘤（HB）发生中枢神经系统（CNS）转移的临床特点、治疗及预后。方法回顾性分析2011年1月至2016年6月首都医科大学附属北京同仁医院收治的12例HB发生CNS转移患儿的临床资料，总结HB发生CNS转移的临床特点、治疗及预后。结果1．临床特点：12例患儿依据术后儿童肿瘤协助组（COG）分期均为Ⅳ期病例，初次就诊时的肝外转移病灶为肺转移12例、骨转移3例、肾上腺转移2例、右心房转移1例、腹腔淋巴结转移1例。HB病理分型中（胎儿＋上皮）混合型最多见（50％，6／12例）。12例均在治疗过程中诊断CNS转移。发生CNS转移为脑转移瘤11例、脊髓转移瘤1例。2．CNS转移的治疗：诊断CNS转移的HB均予以化疗为主的综合治疗。10例诊断CNS转移后给予维持化疗，2例给予化疗＋颅内肿瘤切除术的综合治疗。3．CNS转移HB的预后：12例患儿随访时间7～54个月，中位随访时间20个月。诊断CNS转移后的HB生存期为1—15个月，中位生存时间为3．5个月。其中2例诊断CNS转移后给予颅内肿瘤切除术及维持化疗，随访至2016年6月，生存时间分别为15个月和5个月；另外10例未行手术患儿的生存时间为1—6个月，中位生存时间为3个月。应用乘积极限法绘制CNS转移患儿的生存函数曲线，并进行Log—Rank检验，颅内肿瘤切除术联合化疗患儿的生存期长于未行颅内肿瘤切除手术患儿的生存期（P〈0．05）。结论HB发生CNS转移病例均发生在Ⅳ期的HB病例，均伴肺转移。CNS转移部位以脑转移瘤最多见，为血行性转移途径．脊髓转移1例考虑为局部浸润转移。Ⅳ期HB发生CNS转移的患儿生存期短，预后差，但给予颅内肿瘤切除手术可能延长生存期。Objective To investigate the clinical characteristics, treatment and prognosis of hepatoblastoma （HB） patients with the central nervous system （CNS） metastasis. Methods The clinical data of 12 patients with CNS metastasis who were admitted to Beijing Tongren Hospital, Capital Medical University from January 2011 to June 2016 were retrospectively analyzed. The clinical features, treatment and prognosis of HB patients with CNS metastasis were summarized. Results （1） Clinical features：all 12 patients were diagnosed as stage - 1V patients according to posto- perative Children＇s Oncology Group （ COG ） stage system. The primary positions of extrahepatic metastatic characteris- tics were as follows：lung metastasis in 12 cases, bone metastasis in 3 cases, and adrenal metastasis in 2 cases, right atrial metastasis in 1 case, and abdominal lymph node metastasis in 1 case. Fetal and epithelial mixed type was the most common type in pathologic Pathologic classification of HB （50% ,6/12 cases）. CNS metastasis was detected in all 12 cases during the course of treatment. Brain metastasis were found in 11 cases of CNS imaging features, and 1 case had spinal metastatic tumors. （ 2 ） The treatment for CNS metastasis ： all cases were given chemotherapy - based comprehen- sive treatment after being diagnosed as CNS metastasis of HB. Ten cases of CNS metastasis were treated by maintenance chemotherapy and 2 cases received chemotherapy plus intracranial tumor resection. （3）The prognosis of HB with CNS metastasis ：the follow- up time was from 7 months to 54 months in 12 HB cases with CNS metastasis, and the median follow - up time was 20 months. The survival time of HB was 1 - 15 months, and the median survival time was 3.5 months after the diagnosis of CNS metastasis. Two cases of CNS metastasis were given intracranial tumor resection and chemotherapy and were followed up to June 2016, and survival time was 15 months and 5 months respectively. The sur- vival time of other 10 cases without intracra

关 键 词：肝母细胞瘤 转移 预后 

分 类 号：R735.7[医药卫生—肿瘤]