Abstract
Cholangiocarcinoma developing from the remnant bile ducts following cyst excision for congenital biliary dilatation (CBD) is an increasing problem. There are some reports demonstrating that cholangiocarcinoma develops in approximately 0.7–6.5% of patients who undergo cyst excision; the incidence is 121.5 times higher than that of the general population. The cumulative incidence of cholangiocarcinoma at 15, 20, and 25 years after cyst excision for CBD is reported to be 1.6%, 3.9%, and 11.3%, respectively. Repeated cholangitis, hepatolithiasis, or remnant dilated bile ducts due to inadequate cyst excision have been reported to be risk factors for cholangiocarcinoma following cyst excision. Biliary-enteric anastomosis itself is also reported to be a risk factor for cholangiocarcinoma. The outcomes of treatment for cholangiocarcinoma developing from the remnant bile ducts following cyst excision are unfavorable. The overall cumulative survival rates at 2 and 3 years after treatment were reported to be 32% and 16%, respectively. The median survival time was 15 months. Most patients were diagnosed at the late stage of cholangiocarcinoma. Longer follow-up is needed, even after complete cyst excision, because of the lifelong risk of subsequent cholangiocarcinoma. The regular checkups that include the evaluation of tumor markers (including CEA and CA19-9) and imaging studies, such as abdominal US and CT, are useful for the diagnosis of cholangiocarcinoma developing from the remnant bile ducts.
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Kobayashi, T., Ohashi, T., Sakata, J., Miura, K., Wakai, T. (2018). Cholangiocarcinoma Developing from the Remnant Bile Ducts Following Cyst Excision for Congenital Biliary Dilatation. In: Kamisawa, T., Ando, H. (eds) Pancreaticobiliary Maljunction and Congenital Biliary Dilatation. Springer, Singapore. https://doi.org/10.1007/978-981-10-8654-0_30
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