目的 总结分析儿童胰母细胞瘤(pancreatoblastoma,PBL)治疗期间的各项指标,以提高对PBL的认识.方法 回顾性分析近6 a郑州大学第一附属医院PBL患儿的临床资料,并系统检索国内外胰母细胞瘤相关文献.结果 郑州大学第一附属医院PBL患儿共6例,男3例,女3例,年龄为2~7岁,中位年龄为5岁.6例均可触及腹部肿块,3例腹痛,其余症状如黄疸、贫血、呕吐各1例;肿瘤最大直径为30~160 mm,平均87 mm.2例位于胰头,3例位于胰体尾部,1例位于胰尾部.术后均经病理证实,其免疫组化结果:6例Syn(+)或(灶+),6例CK(+),5例AAT(+),ki-67 (>30%+),3例CD56(+)或(灶+),5例AFP(-),1例AFP(灶+).术后1例拒绝化疗,其余5例行正规化疗,术后随访0.9~3.1 a,1例失访,4例死亡,1例健在.结论 PBL是一种罕见的恶性肿瘤,胰腺各部位均可生长,肿瘤恶性程度高,复发及死亡率较高.目前治疗方案为尽早完整切除瘤体,并辅以化疗.如切除完整并辅以化疗,部分患者可长期生存.%Objective To further learn about pediatric pancreatoblastoma (PBL) by investigating its clinical features, diagnosis and treatment methods and the recent prognosis, etc.Methods A retrospective review of pediatric PBL cases was undertaken within 6 years in the First Affiliated Hospital of Zhengzhou University.Results Six cases of pediatric PBL, 3 males, 3 females, age range was 2 years old to 7 years old, mean age was 5 years old.There were 6 cases of abdominal mass, 3 cases of abdominal pain, 1 case of jaundice, 1 case of anemia, 1 case of vomiting.The maximum diameters of the tumors ranged from 30 mm to 160 mm, and the average diameter was 87 mm.Two cases located in the pancreatic head, 3 cases at the end of the pancreatic body, 1 case in pancreatic tail.The immunohistochemical results: 6 cases of Syn (+) or (range+), 6 cases of CK (+), 5 cases of AAT (+), Ki-67 (>30%+), 3 cases of CD56 (+) or (range+), 5 cases of AFP (-), 1 cases of AFP (range+).One patient refused chemotherapy after operation and five patients received chemotherapy.The follow-up period was 11 months to 37 months.Two patients were lost to follow-up.Three patients died.One patient was alive.Conclusion PBL is a rare malignant tumor, more common in children.The various parts of the pancreas can grow.Because of the high degree of malignancy of the tumor, so its recurrence rate and mortality are high.The preferred treatment regimen is a complete resection of the tumor, followed by chemotherapy.Some patients can survive for a long time after complete resection with chemotherapy.
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