Abstract:

 Objective To analyzes the clinical and pathological characteristics of liver transplantation in2 children with pancreatoblastoma （PB） liver metastasis. Methods Theclinicopathological and immunophenotypic features of 2 children with multiple liver metastasis of PB were analyzed retrospectively. Two cases were detectedand followed up by immunohistochemical method， and the related literatures were reviewed to explore whether liver transplantation can be used as a new treatment for advanced PB in children. Results In the 2 cases of PB， the focus of liver was elevated AFP at the begining， pancreatic mass was found by imaging examination， pathological puncture showed liver metastasis of PB， and there was no obvious remission after chemotherapy. After pancreaticoduodenectomy and liver transplantation， routine pathological examination showed PB with multiple liver metastasis （stage Ⅳ）. Immunophenotype ：tumor cells simultaneously expressed AACT， CK， CK5/6， CD10，β-catenin and neuroendocrine markers （Syn， CgA， etc.）. During the follow-up， the liver function was normal and there was no tumor recurrence and metastasis. Conclusion When liver metastasis occurs in children with advanced PB， the pathomorphological characteristics and immunohistochemical expression of liver metastasis are basically consistent with the primary focus of pancreas. For patients with multiple liver metastasis， better prognosis can be obtained after liver transplantation. 

Key words:

Pancreatoblastoma , Liver metastasis , Liver transplantation , Clinicopathological features , Immunohistochemistry

摘要：

目的 探讨因儿童胰母细胞瘤（pancreatoblastoma，PB）肝转移而进行肝移植的 2 例临床资料的病理学特征。方法 回顾性分析 2 例儿童 PB 多发肝转移的临床病理及免疫表型特征，采用免疫组化法对２例进行检测并随访，探讨肝移植能否作为晚期儿童 PB 治疗的新方案。结果 2 例 PB 均以肝脏病灶为首发伴 AFP 升高，经影像学检查发现胰腺肿物，病理穿刺提示 PB 肝转移，经化疗后无明显缓解，行胰、十二指肠切除及肝移植术后，常规病理检查为 PB 并肝脏多发转移（Ⅳ期）。免疫表型 ：肿瘤细胞同时表达 AACT、CK、CK5/6、CD10、β-catenin 及神经内分泌标记（Syn、CgA 等）。术后随访患者肝功能均正常，无肿瘤复发及转移。结论 儿童 PB 晚期发生肝转移时，肝脏转移灶的病理形态学特征及免疫组化表达与胰腺原发灶基本一致，对于肝脏多发转移的患者，经过肝移植可以获得较好的预后。 

关键词:

胰母细胞瘤 , 肝转移 , 肝移植 , 临床病理特征 , 免疫组织化学