小儿胰母细胞瘤的诊断和治疗  

The Diagnosis and Treatment of Pancreatoblastoma

作　　者：祝秀丹[1,2] 李家驹[1,2] 宋桂云[1,2] 罗平风[1,2] 

机构地区：[1]首都医科大学附属北京儿童医院外科 [2]大连市儿童医院外科

出　　处：《中华小儿外科杂志》1998年第1期39-41,共3页Chinese Journal of Pediatric Surgery

摘　　要：目的：介绍小儿胰母细胞瘤的诊断治疗经验。临床资料：手术病理诊断８例。年龄１～８岁。男６例，女２例。多以腹块（６／８）、腹痛（４／８）就诊。可误诊为腹膜后神经母细胞瘤、畸胎瘤及黄疸性肝炎。５例ＡＦＰ有轻～中度升高（２０．５６～５９８．８７μｇ／Ｌ），Ｘ线平片及ＣＴ均可见不同程度钙化。肿瘤部位：胰头３例、胰体３例、胰尾２例。局部切除５例、扩大切除（Ｗｈｉｐｐｌｅ术）１例、活检２例。术后分期：Ⅰ、Ⅱ期３例，Ⅲ期２例，Ⅳ期３例。组织学特点：分界清，略分叶，肉眼见有包膜，光镜下与胚胎期８周时的胰胚基相似。治疗结果：本组６例化疗者均生存，最长者已８年。１例Ⅲ期患儿活检后化疗肿瘤消失、ＡＦＰ降至正常，无瘤生存至今已４年。１例Ⅳ期患儿化疗３年至今带瘤生存。化疗药物以长春新碱、环磷酰胺、阿霉素为主，复发者加放疗及顺铂。结论：胰母细胞瘤影像学检查有非特异性钙化。血ＡＦＰ值可作为瘤标用于诊断及随访。肿瘤对化疗敏感，难以一期切除者活检后先化疗，再酌情施行根治性手术。其预后比成人胰母细胞瘤及胰腺癌要好，应积极治疗。Objective: Pancreatoblastoma (PB) is a rare pancreatic malignant tumor in childhood. The experience on the diagnosis and treatment of 8 cases is reported. Methods: From 1989 to 1997, 8 cases (male 6, female 2) aged from 1-8 years were operated on, and the diagnosis were confirmed pathologically. The presentation was abdominal mass (6/8) and pain (4/8). Five case had mild to moderate increase in AFP (20.56-598.87μg/L). The CT scan and Xray film all demonstrated calcification. The focus of tumor was in the head (3), body (3) and tail (2) of pancreas. Distant metastasis were found in liver (3/8), spleen (1/8) and pelvis (1/8). The operation included local resection on 5, Whippls procedure 1 and biopsy 2. The main chemotherapy were vincristin, cyclophosphamide and Adriamycin. Results: Three cases (stage Ⅰ-Ⅲ) survived without PB with a followup of 1-8yrs, the other 3 (stage Ⅲ、 Ⅳ) have lived with PB from 2 mons to 3 yrs after operation. Conclusions: 1. CT scan and Xray examination of PB may demonstrate unspecialised calcification; 2. A positive AFP may serve as the tumor marker; 3. Chemotherapy is effective; 4. The prognosis of PB is better in children than in adults.

关 键 词：胰母细胞瘤 药物疗法 放射疗法 儿童 诊断 

分 类 号：R735.9[医药卫生—肿瘤]