儿童胃肠道恶性肿瘤的诊断和外科治疗

The diagnosis and surgical treatment of malignant gastrointestinal tumors in children

目的 探讨儿童胃肠道恶性肿瘤的诊断和治疗要旨.方法 对1996年1月至2008年5月间收治的29例胃肠道恶性肿瘤患儿的病例资料,包括肿瘤的发生部位、肿瘤类型、临床表现、治疗效果及预后进行回顾性分析.结果 患儿男26例,女3例.初诊时年龄1岁10个月～14岁,中位年龄lO岁.胃肿瘤5例,包括间质瘤1例、恶性淋巴瘤2例、内胚窦瘤1例和P-J综合征恶变1例.肠道肿瘤24例,包括恶性淋巴瘤17例、结肠癌4例和阑尾类癌3例.临床主要表现为腹痛、呕吐、发热、消化道出血、消瘦等.外科并发症主要包括穿孔、内瘘、梗阻、套叠、出血.24例接受（胃）肠切除肠吻合术,5例接受活检.2例结肠癌患儿术后半年至1年复发.17例患儿存活至今,6例失访,6例死亡.结论 儿童胃肠道恶性肿瘤发病率低,临床表现无特异性,术前诊断较困难,在急腹症和胃肠道疾患的诊断中应该注意肿瘤的鉴别诊断,强调运用各种影像学和内镜技术辅助诊断;治疗采用手术切除结合化学治疗;预后与恶性肿瘤的病理本质有关.

Objective To investigate the essential points of the diagnosis and the surgical treatment of malignant gastrointestinal tumors in children. Methods Clinical data involved the sites and pathological types of the tumors, clinical manifestation, effective treatment and prognosis were retrospectively analyzed in 29 consecutive patients reffered to Beijing Children＇s Hospital between January 1996 and May 2008. Results Of 29 patients, 26 were males and 3 were females （aged from 1 year and 10 month to 14 years, mean age of 10 years）. Gastric tumor was found in 5 eases （interstitialoma in 1, lymphoma in 2, endodermal sinus tumor in 1 and P-J syndrome canceration in 1）, whereas intestinal tumor was found in 24 cases （malignant lymphoma in 17, colon carcinoma in 4 and earcinoid of appendix in 3）. The main clinical manifestations were abdominal pain, emesia, fever, alimentary tract hemorrhage and emaciation. Surgical complication involved perforation, internal fistula, obstruction, invaginate and hemorrhage. Twent^four patients underwent （gaster） intestine resection and anastomosis. Biopsy was performed in 5 patients. Two patients with colonic carcinoma relapsed 6 months to 1 year after operation. Of 29 patients, 17 patients survived, 6 patients missed the follow-up and 6 patients died. Conclusions Pediatric malignant gastrointestinal tumor is rare. The unspecific presentation makes the diagnosis difficult. In diagnosis of acute abdomen and gastrointestinal tract diseases, the possibility of malignant tumor should be reminded. The imaging and endoscopic examination are very important to diagnosis. The surgical resection or combined with chemotherapy are mainly used in treatment of tumors. The prognosis is related to the pathological nature of the tumor.