摘要：
Objective To analyze the clinical characteristics, treatment and prognostic factors of children with pancreaticoblastoma ( PBL) . Methods The clinical characteristics, treatment and outcome of 10 children with PBL admitted to the First Affiliated Hospital of Zhengzhou University from January 2008 to March 2018 were retrospectively analyzed, and the related factors of prognosis were analyzed. Results Ten children were with PBL, aged 2-7 years, with the median age of 5 years, and the ratio of male to female was 1:1. In the initial diagnosis, 9 cases had palpable abdominal mass, including 4 cases of abdominal pain, 2 cases of diarrhea, 1 case of jaundice, 1 case of anemia and 1 case of vomiting symptoms. There were 3 cases of pancreatic head tumor, 5 cases were in body and tail of pancreas, and 2 cases were in tail of pancreas. Postoperative immunohistochemical results showed 6 cases of Sny (+) or ( leison+) , 10 cases of cytokeratin (+) , 8 cases of α -antitryptase ( +) , 9 cases of ki-67 ( > 30％+) , 1 case of ki-67 (30％) , 3 cases of CD56 (+) or ( leison+) , 9 cases of α - fetoprotein (-) , 1 case of α-fetoprotein ( leison+) . One case did not receive chemotherapy and died after 5 months. The remained 9 cases received chemotherapy after operation. During 0. 9 to 5. 0 years follow-up, 4 cases of 9 patients survived without tumors, 1 case lost follow-up and 4 cases died. Conclusions PBL is a rare malignant tumor, which can occure in each part of the pancreas. The malignancy, recurrence and mortality of PBL are high. Complete resection and progression of tumors are important factors affecting prognosis. Chemotherapy can remove some of the tumors that cannot be surgically removed. Lomplete excision adjuvant with chemotherapy can bring some patients a long time of survival.%目的 分析胰母细胞瘤(PBL)患儿的临床特点、治疗及影响预后的相关因素.方法 回顾性分析2008年1月至2018年3月郑州大学第一附属医院收治的10例PBL患儿的临床特点、治疗方法及转归,进行相关因素预后分析.结果 10例PBL患儿,男:女为1:1,年龄2～7岁,中位年龄5岁.初诊时可触及腹部肿块9例,腹痛4例,腹泻2例,黄疸、贫血、呕吐症状各1例;胰头部肿瘤3例,胰体尾部5例,胰尾部2例.术后免疫组化结果显示:6例突触素(+)或(灶+),10例细胞角蛋白(+),8例α-抗胰蛋白酶(+),9例ki-67(>30％+),1例ki-67(30％),3例CD56(+)或(灶+),9例甲胎蛋白(-),1例甲胎蛋白(灶+).术后1例未化疗,5个月后死亡;余9例行术后化疗,术后随访0.9～5.0年,4例无瘤生存至今,1例失访,4例死亡.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,肿瘤是否完全切除、是否进展等是影响预后的重要因素.化疗可使部分无法手术切除的肿瘤达到可切除的状态.完整切除瘤体并辅以化疗,部分患者可长期生存.
