探讨成人胆总管囊肿癌变的单中心外科诊疗经验。

回顾性分析广西医科大学第一附属医院2013年1月至2025年6月收治的77例成人胆总管囊肿患者的临床资料。回顾性研究免除知情同意，符合医学伦理学规定。其中男21例，女56例；年龄18~76岁，中位年龄37岁。统计胆总管囊肿癌变病例，分析相关临床数据。生存分析采用Kaplan-Meier法和Log-rank检验。

77例胆总管囊肿患者中发生癌变10例，癌变发生率为13%（10/77），其中男3例，女7例；年龄26~72岁，中位年龄40岁。其中行根治性胰十二指肠切除术7例（根治性手术组），手术时间398~593 min，中位手术时间为510 min；术中出血量300~1 400 ml，中位出血量800 ml；术后并发症Clavien-Dindo分级Ⅲa及以上2例，无围手术期90 d内死亡。姑息手术3例（姑息治疗组），其中1例行肝转移瘤活检术，1例行胆道支架植入术，1例行胆总管囊肿切除+姑息性胆肠内引流术。随访截至2025年6月30日，根治性手术组术后随访时间1~146个月，中位生存期16个月，随访期间2例死于术后12、18个月，1、2、3年生存率分别为83.3%、62.5%、62.5%。姑息治疗组术后随访时间1~6个月，中位生存期5个月，随访期间3例死于术后1、5、6个月；1、2、3年生存率均为0。Kaplan-Meier生存分析显示，根治性手术组患者中位生存期明显长于姑息治疗组（χ²=8.64，P=0.003），生存获益更明显。

成人胆总管囊肿癌变的首要预防策略是早期诊断并施行彻底的胆总管囊肿切除术，尤其强调对胆胰汇合部的彻底处理，尽可能切除胰腺段胆总管囊肿壁，以减少残留胆总管囊肿；胆总管囊肿切除术后必须终身规律随访；一旦诊断为胆总管囊肿癌变，争取行胰十二指肠切除术。

To investigate the single-center experience in the surgical diagnosis and treatment of malignant transformation of choledochal cysts in adults.

The clinical data of 77 adult patients with choledochal cysts admitted to the First Affiliated Hospital of Guangxi Medical University from January 2013 to June 2025 were retrospectively analyzed. As a retrospective study, the requirement for informed consent was waived, and the study complied with medical ethical requirements. There were 21 males and 56 females, aged 18-76 years, with a median age of 37 years. Cases of malignant transformation of choledochal cysts were identified, and the relevant clinical data were analyzed. Survival analysis was performed using the Kaplan-Meier method and log-rank test.

Malignant transformation occurred in 10 of the 77 patients with choledochal cysts, with an incidence of 13% (10/77). Among them, there were 3 males and 7 females, aged 26-72 years, with a median age of 40 years. Seven patients underwent radical pancreatoduodenectomy (radical surgery group). The operative time ranged from 398 to 593 min, with a median of 510 min; intraoperative blood loss ranged from 300 to 1,400 ml, with a median of 800 ml. Two patients developed postoperative complications of Clavien-Dindo grade Ⅲa or higher, and no deaths occurred within 90 d after surgery. Three patients underwent palliative surgery (palliative treatment group), including liver metastasis biopsy, biliary stent implantation, and choledochal cyst resection combined with palliative internal biliary-enteric drainage in one case each. Follow-up ended on June 30, 2025. The follow-up duration of the 7 patients after radical surgery ranged from 1 to 146 months, with a median survival time of 16 months. During follow-up, two patients died at 12 and 18 months after surgery, respectively. The 1-, 2-, and 3-year survival rates were 83.3%, 62.5%, and 62.5%, respectively. The follow-up duration after palliative surgery ranged from 1 to 6 months, with a median survival time of 5 months. During follow-up, all 3 patients died at 1, 5, and 6 months after surgery, respectively; the 1-, 2-, and 3-year survival rates were all 0. Kaplan-Meier survival analysis showed that the median survival time in the radical surgery group was significantly longer than that in the palliative treatment group ( χ² = 8.64, P = 0.003), indicating a more pronounced survival benefit.

The primary preventive strategy for malignant transformation of choledochal cysts in adults is early diagnosis and complete excision of the choledochal cyst. Particular emphasis should be placed on complete management of the pancreaticobiliary junction and maximal removal of the intrapancreatic cyst wall to reduce residual choledochal cyst tissue. Lifelong regular follow-up is required after choledochal cyst excision. Once malignant transformation of a choledochal cyst is diagnosed, pancreatoduodenectomy should be attempted whenever feasible.