小儿胰腺肿瘤较为少见,仅占儿童肿瘤的0.6%～0.8%。发病率位于胰管畸形引起的胰腺炎和胰腺外伤之后,居小儿胰腺疾病的第三位。肿瘤多发生于学龄期或青春前期儿童,男孩多于女孩。恶性肿瘤占80%以上,以胰母细胞瘤、乳头状囊腺癌多见,其次为胰腺癌、胃泌素瘤和其他胰腺肉瘤。良性肿瘤以乳头状囊腺瘤最多见,但恶变率甚高。与其他小儿恶性肿瘤的发病特点相似,小儿胰腺肿瘤的发病规律符合遗传性肿瘤的特点,生物特性多支持 Kundson 的二次体细胞突变学说,近年应用组织形态和组化免疫等手段证实最常见的胰母细胞瘤和乳头状囊腺瘤的发生都与胰腺个体发育异常 Pediatric pancreatic tumors are relatively rare, accounting for only 0.6% to 0.8% of children with tumors. The incidence is located in the pancreatic duct caused by pancreatitis and pancreatic trauma, ranking third in children with pancreatic disease. Tumors occur mostly in school-age or adolescent children, boys than girls. Malignant tumors accounted for more than 80%, with pancreatoblastoma, papillary cystadenocarcinoma more common, followed by pancreatic cancer, gastrinoma and other pancreatic sarcoma. Benign tumor papillary cystadenoma most common, but the high rate of malignancy. Similar to the incidence of other pediatric malignancies, the incidence of pancreatic tumors in children is in line with the characteristics of hereditary tumors. Kunson’s second somatic mutation is more likely to be supported by biological characteristics. In recent years, histological and histochemical findings confirm the most common Pancreatoblastoma and papillary cystadenoma occur in individuals with developmental abnormalities in the pancreas