Shimamura Tsuyoshi, Watanabe Masaaki, Koshizuka Yasuyuki, Goto Ryoichi, Kawamura Norio, Orimo Tatsuya, Kamachi Hirofumi, Kamiyama Toshiya, Mitsuhashi Tomoko, Hibi Taizo, Taketomi Akinobu
Division of Organ Transplantation, Hokkaido University Hospital, Sapporo, Japan.
Department of Transplant Surgery, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Surg Case Rep. 2024 Dec 4;10(1):274. doi: 10.1186/s40792-024-02068-5.
In patients with pancreaticobiliary maljunction complicated by congenital biliary dilatation, the pancreatic enzyme flows back into the bile, leading to bile duct carcinogenesis. Although the biliary tract resection and reconstruction is well documented to decrease the rate of malignancy, cancer occurrence has been reported in the residual intrahepatic or intrapancreatic bile duct, even after resection. We report a case of multiple biliary tract cancers in the liver complicated by congenital biliary dilatation, whose tumor lesions were resected en bloc without disconnecting the biliary tract by simultaneous pancreatoduodenectomy and living donor liver transplantation.
A 27-year-old woman presented with epigastric discomfort. Examination indicated multiple biliary tract cancers complicated by congenital biliary dilatation. Computed tomography scan revealed three papillary tumors in the right hepatic duct with increased F-FDG accumulation on positron emission tomography. Contrast-enhanced ultrasound revealed another lesion in the left hepatic duct. Adenocarcinoma cells were detected using bile and choledochal brush cytology. Tumors resection by right lobectomy or trisegmentectomy of the liver and extrahepatic bile duct resection indicated a high risk of postoperative liver failure; the residual liver volumes were calculated only 277 ml or 176 ml, respectively. In addition, tumor recurrence owing to bile leakage during the surgery and carcinogenesis from the remaining bile duct were concerned. Pancreatoduodenectomy was performed without disconnecting the biliary tract, and the tumors were resected en bloc with the whole liver. The left lobe liver graft from the husband was then transplanted. After 5 years of adjuvant treatment with tegafur/gimeracil/oteracil potassium, she remained in remission eight and half years after the surgery.
Given the mechanism and development of cancer in the congenital biliary dilatation, simultaneous pancreatoduodenectomy and liver transplantation may be considered, especially in the case of young patients.
在胰胆管合流异常合并先天性胆管扩张的患者中，胰酶反流至胆汁中，导致胆管癌变。尽管胆管切除重建术能有效降低恶性肿瘤发生率，但即使在切除术后，肝内或胰内残留胆管仍有癌变报道。我们报告一例先天性胆管扩张合并肝脏多发胆管癌患者，通过同步胰十二指肠切除术和活体肝移植术，在不离断胆管的情况下将肿瘤病灶整块切除。
一名27岁女性因上腹部不适就诊。检查发现为先天性胆管扩张合并多发胆管癌。计算机断层扫描显示右肝管有三个乳头状肿瘤，正电子发射断层扫描显示氟代脱氧葡萄糖摄取增加。超声造影显示左肝管另有一个病灶。通过胆汁和胆管刷检细胞学检查发现腺癌细胞。行右半肝或三段肝切除及肝外胆管切除，术后肝衰竭风险高；剩余肝体积分别仅计算为277ml或176ml。此外，还担心手术中胆汁漏导致肿瘤复发以及残留胆管发生癌变。在不离断胆管的情况下进行了胰十二指肠切除术，将肿瘤与整个肝脏整块切除。然后移植了来自其丈夫的左叶肝脏。在接受替加氟/吉美嘧啶/奥替拉西钾辅助治疗5年后，术后八年半仍处于缓解期。
鉴于先天性胆管扩张症中癌症的发生机制和发展情况，可考虑同步进行胰十二指肠切除术和肝移植，尤其是对于年轻患者。
