儿童胰母细胞瘤14例临床分析

Report of 14 cases with pancreatoblastoma

目的分析胰母细胞瘤患儿的临床特点、治疗情况及影响预后的因素。方法回顾性分析2003年4月至2013年7月上海交通大学附属上海儿童医学中心、重庆医科大学附属儿童医院、首都医科大学附属北京儿童医院、中山大学肿瘤防治中心4家医院收治的14例胰母细胞瘤患儿，总结分析其临床特点、手术及化疗情况，并进行预后分析。结果14例患儿中位诊断年龄6．5岁（0．6—11岁）。初诊时4例为局部肿块，9例为局部肿瘤伴有胰外组织或临近脏器浸润，1例伴有肺、肝脏的远处转移。10例患儿中有8例伴有甲胎蛋白升高（66—54000μg/L）。8例先行手术切除，6例行肿瘤活检术，其中4例在接受化疗4—6个疗程后行二次手术。除2例患儿分别在1和2个疗程后放弃治疗外，其余12例均按计划完成化疗。中位疗程数6（2～12）个。1例接受射频消融术。治疗结束时9例患儿达到缓解，1例在停药5个月后肝转移性复发，其余患儿中位随访时间为26（16～140）个月。除去2例放弃治疗者，本组患儿2年无事件生存率（66．7±13．6）％。将各临床特征进行单因素预后分析，肿块无法完整切除（x2=6．663，P=0．010），治疗过程中出现进展或转移（x2=14．927，P=0．000），治疗结束时未完全缓解（x2=12．293，P=0．000）是影响预后的危险因素。结论手术能否完全切除肿瘤、治疗结束时是否完全缓解、病程中是否进展是影响预后的重要因素。化疗可使部分初诊时不可切除的肿瘤达到可切除状态，并改善预后。

Objective To analyze clinical features, outcomes and correlative prognostic factors of the disease. Method Totally 14 newly diagnosed children with pancreatoblastoma at Shanghai Children＇s Medical Center, Children＇s Hospital of Chongqing Medical University, Sun Yat-sen University Cancer Center and Beijing Children＇s Hospital were enrolled into this study between April 2003 and July 2013. The clinical features, surgery, chemotherapy protocol and prognostic factors of patients were retrospectively analyzed. Result The median age at diagnosis was 6. 5 years （0.6 to 11 years）. Four cases had local tumor, 9 cases had local invasion, and in l case lung and liver metastasis was found at diagnosis. Increased serum ot-fetoprotein were found in 8 cases （66 -54 000 μg/L）. In 8 patients the tumor was resected at first. The remaining 6 patients had biopsy and in four of them the tumor was completely resected after 4 - 6 cycles of chemotherapy. Except for 2 patients who gave up treatment, all the other patients fulfilled the comprehensive treatment including surgery and chemotherapy. One patient received radiofrequency ablation. The median follow-up period was 26 months（ 16 - 140 months）. The estimated 2 year event free survival was （66. 7 ± 13.6） %. On univariate analysis, non-resectable tumor（ X2 = 6. 663, P = 0. 010 ）, progression and metastases during treatment （X2 = 14. 927, P = 0. 000 ）, poor response to treatment （x2 = 12. 293, P = 0. 000 ） were independent predictors for poor prognosis. Conclusion Complete resection and complete remission after treatment were exeel｜ent prognostic factors. Some initially unresectabie disease seems to be resectable after adjuvant chemotherapy, which may be a key point of treatment strategy.