伴肝转移的胰母细胞瘤临床特点及治疗结果

Clinical features and treatment outcome of children with liver metastatic pancreatoblastoma

目的总结伴肝转移的儿童胰母细胞瘤(PB)的临床特点,并进行疗效及并发症分析。方法回顾性分析2002—2021年间北京儿童医院收治的11例伴肝转移的胰母细胞瘤患儿。总结其临床特征、实验室检查、肝转移灶数量及大小、病理结果、治疗方案,并进行疗效及并发症分析。结果共纳入11例转移性PB患儿,其中男7例、女4例,中位诊断年龄5岁。均为多发肝转移。腹痛8例、黄疸2例、呕吐2例。10例伴血清甲胎蛋白(AFP)升高,确诊时AFP中位数为426.87ng/ml(2.08~53521ng/ml)。3例患儿单纯肝转移,8例患儿伴其他部位多发转移(5例脾静脉瘤栓、4例门静脉瘤栓、3例肠系膜上静脉瘤栓、3例瘤周淋巴结转移,1例骨转移)。门脉海绵样变2例,伴消化道穿孔1例,2例患儿伴有肿瘤破裂。新辅助化疗及手术切除作为主要治疗方案。中位随访时间为7个月,存活4例,复发1例,死亡4例,失访2例。结论伴肝转移的胰母细胞瘤临床表现无特异性,以腹痛为主。除肝转移外,常伴多发转移,如周围静脉瘤栓及瘤周淋巴结转移。多数患儿出现AFP明显升高,原发灶位于胰头及多发转移可能影响患儿预后。新辅助化疗及手术切除作为最有效的治疗方案可改善患儿预后。

Objective To analyzes the clinical characteristics of children with liver metastatic pancreatoblastoma and evaluates its therapeutic effect and prognosis.Methods From May 2002 to May 2021,11 children with liver metastatic pancreatoblastoma were diagnosed by pathology.Retrospective analysis of the children on clinical features,laboratory examinations,number and size of liver metastases,pathological results,treatment plan,curative effect and complications were performed.Results The 11 children with metastatic PB were confirmed by pathology.There were 7 males(64%)and 4 females(36%).The median age of diagnosis was 5 years old.All of the children were multiple liver metastases and without specific manifestations at the time of onset.The most common symptoms were abdominal pain(n=6),jaundice(n=2)and vomiting(n=2).10 cases were accompanied by elevated serum alpha fetoprotein(AFP)and the median AFP was 426.87 ng/ml(2.08-53521ng/ml)at the time of diagnosis.In this study,3 patients had liver metastasis alone.8 patients had other metastases,including 5 cases of portal vein tumor thrombus,4 cases of spleen vein tumor thrombus,3 cases of superior mesenteric vein thrombus,3 cases of lymph node metastases around tumor and 1 case of bone metastasis.Several cases had complications at the time of diagnosis,including 2 cases of portal cavernous transformation,1 case of gastrointestinal perforation,and 2 cases of children with tumor rupture.Neoadjuvant chemotherapy and surgical resection are the most treatment methods.The median follow-up time of 11 patients was 7 months,4 cases survived,1 case relapsed,4 cases died and 2 cases lost follow-up.Conclusions Liver metastatic pancreatoblastoma lacks specific manifestations,and abdominal pain is the most common symptom.In addition to liver metastases,most children of multiple metastases may be accompanied with peripheral vein tumor thrombosis and lymph node metastasis.AFP is significantly increased in most children.The primary tumor located in the head of pancreas and multiple metastases may affect the prognosis of children.Neoadjuvant chemotherapy and surgical resection are the most effective treatment options,which can improve the prognosis of children.