儿童结肠癌三例并文献复习

Pediatric Colon Carcinoma: a report of 3 cases and literature review

目的报告3例儿童结肠癌(pediatric coloncarcinoma,PCC),回顾相关文献了解预后影响因素以提高该病的诊疗水平。方法回顾性分析首都医科大学附属北京儿童医院2006年8月至2017年8月收治的3例结肠癌患儿临床资料。3例均为男孩,平均年龄12岁4个月:病例1急诊入院,临床表现腹痛、发热及排便困难,B型超声检查提示结肠穿孔;余2例患儿临床表现为腹痛、便血,B型超声、腹部CT及结肠镜检查考虑为降结肠肿物。检索Pubmed和Cochrane Library数据库中截至2018年4月1日关于儿童结肠癌的相关文献对预后影响因素进行分析。结果对病例1行回肠造痿术,术后出现中毒性休克放弃治疗,于术后1周死亡,病理检查结果为印戒细胞癌,临床分期ⅣC期。对病例2和病例3行左半结肠癌扩大切除术,病理检查结果分别为降结肠印戒细胞癌和腺癌,临床分期分别为ⅢC期和ⅢB期。所有病例术后均采用FOLFOX化疗方案,病例2化疗10个疗程,术后半年复发,1年半死亡;病例3化疗3个疗程(结合中药辅助治疗),术后8个月未见复发。通过文献检索,共检索到40篇文献,对于确诊后生存时间超过1年的患儿情况进行分析,发现结肠癌患儿1年生存率<50%,肿物位置及临床分期对患儿生存率有较大的影响。结论儿童结肠癌是临床罕见的肿瘤,漏诊误诊率高,目前主要依靠增强CT和结肠镜进行诊断。主要治疗方法是手术完整切除加术后化疗,部分晚期患儿联合靶向治疗。患儿总体预后不佳,早期发现并积极治疗可延长生存期。

Objective To report 3 cases of pediatric colon carcinoma (PCC), review the relevant literatures and improve its clinical awareness. Methods Clinical data were retrospectively reviewed for 3 PCC cases from August 2006 to August 2017 at Beij ing Children Hospital. They were all boys with a median age of 12. 3 years at diagnosis. Case 1 presented with abdominal pain, fever and constipation and was diagnosed as colonic perforation by type B ultrasonography. The remaining two cases had abdominal pain and hematochezia and were diagnosed as descending colon mass by type B ultrasonography, computed tomography (CT) and colonoscopy. Literatures related to PCC were retrieved from Pubmed and Cochrane Library databases until April 1, 2018 for analyzing the prognostic factors. Results Case 1 died within one week due to toxic shock after emergent ileostomy and it was confirmed as signet-ring cell carcinoma by postoperative pathology. The clinical stage was ⅣC. Another 2 cases received FOLFOX chemotherapy after expanded resection for left-sided colon cancer. Pathological examination showed signet ring cell carcinoma and adenocarcinoma respectively. The signet-ring ⅢC patient received 10 sessions of chemotherapy, recurred within 6 months and died within 1. 5 years. Another ⅢB boy had no recurrence after 3 sessions of chemotherapy plus Chinese herbal auxiliary treatment over 8 months postoperatively. A total of 40 literatures were retrieved. One-year survival rate of PCC was under 50%. Tumor location and clinical stage affected the prognosis of PCC. Conclusions As a rare pediatric tumor with a high misdiagnostic rate and a poor prognosis, PCC is definitely diagnosed by CT and colonoscopy. Its major treatment is complete surgical resection plus postoperative chemotherapy. Combined targeted therapy is necessary for advanced PCC patients. Early diagnosis and aggressive treatment are beneficial for improving its overall survival.