Abstract
The incidence of bile duct cancer developed in the patients with congenital choledochal cysts (CC) with a history of reparative surgery is not well known. We experienced a case developed choleductal cancer 45 years after reparative surgery. She underwent reparative surgery with cyst excision and hepatic bile duct duodenal anastomosis at 1 year of age. She developed the symptoms of jaundice, anorexia, and dull pain in the right upper part of the abdomen at 47 years of age. The carcinoma arose from the dilated proximal bile duct anastomosed with the duodenum. Cholestasis and regurgitation of duodenal fluids seemed to have influenced the development of cancer in this patient. We additionally reviewed seven cohort studies concerning the incidence of biliary carcinoma after surgery for congenital choledocal cysts. The incidence of biliary cancer developed after surgery was 2.2 ± 2.5 (ranged 0–6.5)%. We also reviewed 33 Japanese case reports cited in Japan MEDLINE from 1986 to 2015. Regarding the CC types according to the Totani’s classification, 12 were type I and 14 type IVa, 1 was type II and 6 were unknown type. The rate of coexistence of pancreaticobiliary maljunction (PBM) was 92%, (22/24; other 9 cases were not documented), and biliary cancer arose from the proximal stump of the reparative surgery in 68% (17/25) and from the distal stump in 32% (8/25) of cases. These findings suggested that the reflux of pancreatic juice due to PBM only partially explained the carcinogenesis of CCs. Intensive follow-up of such patients throughout their lives is necessary to avoid cancer death even after standard reparative surgery.
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Ikegame, K., Takano, A., Watanabe, H. et al. Biliary cancer developed after the reparative surgery for congenital choledochal cyst: a case report and review of the literature. Int Canc Conf J 6, 43–49 (2017). https://doi.org/10.1007/s13691-016-0270-x
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DOI: https://doi.org/10.1007/s13691-016-0270-x