Cholangiocarcinoma (CCA) is a rare but lethal tumor that arises from the intrahepatic, perihilar, or extrahepatic bile ducts. Complete surgical resection remains the only chance at long-term survival. Unfortunately, most cases of CCA are clinically silent until late in the disease process, and, combined with the lack of effective screening tests, many CCAs present as unresectable tumors. CCA workup typically includes a multiphasic chest, abdominal, and pelvic imaging, liver function tests, and tumor markers (CEA, CA 19-9). Tissue diagnosis is encouraged but not always necessary. In certain situations, esophagogastroduodenoscopy, colonoscopy, and mammography are recommended. If resectable, intrahepatic CCAs and perihilar CCAs require a hepatectomy ranging from a wedge resection to an extended hepatectomy with reconstruction depending on the location and tumor size. In certain specialized centers, portal vein and hepatic artery reconstruction can be performed with good outcomes and acceptable morbidity. For resectable extrahepatic CCAs, a pancreaticoduodenectomy is recommended. Traditionally, few effective adjuvant options have existed for patients after surgery. However, recent randomized controlled trials support the use of either adjuvant chemotherapy or chemoradiation therapy after surgical resection. In select patients, intra-arterial therapy options such as transarterial chemoembolization, hepatic artery infusion therapy, or yttrium-90 radioembolization, as well as liver transplant, are effective treatment modalities. Improved surgical techniques, regionalization of care to high-volume centers, and appropriate application of preoperative optimization techniques have safely expanded the candidates of potentially resectable patients and improved patient outcomes.

(Hewitt, D. B., et al. – Hepatoma Research, November 2021)

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