Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
J Gastroenterol Hepatol. 2013 Feb;28(2):243-7. doi: 10.1111/j.1440-1746.2012.07260.x.
The aim of this study was to elucidate the risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts.
A retrospective analysis of 94 patients who had undergone cyst excision for congenital choledochal cysts was conducted. The median age at the time of cyst excision and median follow-up time after cyst excision were 7 years and 181 months, respectively.
Biliary tract cancer developed in four patients at 13, 15, 23, and 32 years after cyst excision. The cumulative incidences of biliary tract cancer at 15, 20, and 25 years after cyst excision were 1.6%, 3.9%, and 11.3%, respectively. The sites of biliary tract cancer were the intrahepatic (n = 2), hilar (n = 1), and intrapancreatic (n = 1) bile ducts. Of the four patients with biliary tract cancer after cyst excision, three patients underwent surgical resection and one patient received chemo-radiotherapy. The overall cumulative survival rates after treatment in the four patients with biliary tract cancer were 50% at 2 years and 25% at 3 years, with a median survival time of 15 months.
The risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts seems to be relatively high in the long-term. The risk of biliary malignancy in the remnant bile duct increases more than 15 years after cyst excision. Despite an aggressive treatment approach for this condition, subsequent biliary malignancy following cyst excision for congenital choledochal cysts shows an unfavorable outcome.
本研究旨在阐明接受胆囊肿切除手术治疗的先天性胆总管囊肿患者发生后续胆道恶性肿瘤的风险。
对 94 例行胆囊肿切除手术治疗的先天性胆总管囊肿患者进行回顾性分析。胆囊肿切除时的中位年龄和胆囊肿切除后中位随访时间分别为 7 岁和 181 个月。
胆肠肿瘤在胆囊肿切除后 13、15、23 和 32 年分别在 4 例患者中发展。胆囊肿切除后 15、20 和 25 年的胆道恶性肿瘤累积发生率分别为 1.6%、3.9%和 11.3%。胆道恶性肿瘤的部位为肝内（n=2）、肝门（n=1）和胰内（n=1）胆管。胆囊肿切除后发生胆道恶性肿瘤的 4 例患者中，3 例行手术切除，1 例行放化疗。4 例胆道恶性肿瘤患者治疗后的总累积生存率分别为 2 年时 50%，3 年时 25%，中位生存时间为 15 个月。
接受胆囊肿切除手术治疗的先天性胆总管囊肿患者在长期随访中似乎存在较高的继发胆道恶性肿瘤风险。胆囊肿切除后 15 年以上，残留胆管发生胆道恶性肿瘤的风险增加。尽管对这种疾病采取了积极的治疗方法，但先天性胆总管囊肿切除术后的继发胆道恶性肿瘤仍表现出不良的预后。
