儿童胰母细胞瘤6例报道

Diagnosis and treatment analysis of pancreatoblastoma in 6 children

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, diag- nosis and treatment methods and the recent prognosis, etc. Methods A retrospective review of pediatric PBL cases was under- taken within 6 years in the First Affiliated Hospital of Zhengzhou University. Results Six cases of pediatric PBL, 3 males, 3 fe- males, 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 ab- dominal 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 ram. Two cases located in the pancreatic head, 3 cases at the end of the pan- creatic body, 1 case in pancreatic tail. The immunohistochemical results ： 6 cases of Syn （ ＋ ） or （ range ＋ ）, 6 cases of CK （ ＋ ）, 5 cases ofAAT （＋）, 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 ma- lignancy 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.