Episode three organizes cholangiocarcinoma anatomy first, biology second, because where the tumor sits dictates the operation and the transplant path. Intrahepatic disease is resected when possible and is a transplant contraindication over two centimeters, distal disease gets a Whipple, and perihilar disease is resected when resectable with a narrow unresectable subset going through a neoadjuvant-then-transplant protocol. The imaging is delayed enhancement without washout, the mirror image of HCC, because the fibrous stroma traps contrast rather than letting it run through. CA 19-9 is a trend rather than a yes-or-no and is unusable in Lewis-negative patients, so sclerosing cholangitis patients with a new dominant stricture get FISH on the brushings when cytology fails them. Topics covered Three anatomic subtypes and their operations Perihilar subclassification and hepatectomy extent Delayed enhancement versus HCC washout Shared inflammatory risk factors CA 19-9 interpretation and Lewis-negative patients The indeterminate stricture and FISH Perihilar transplant protocol First-line systemic therapy FGFR2 and IDH1 targeted subtypes Key decisions Location dictates the operation: intrahepatic tumors come out with the piece of liver, perihilar tumors need a hemihepatectomy plus extrahepatic bile duct plus Roux-en-Y, and distal tumors need a pancreaticoduodenectomy. Intrahepatic cholangiocarcinoma takes up contrast progressively in the venous and delayed phases with no washout, the opposite of HCC, because the dense fibrous stroma traps and slowly releases contrast. CA 19-9 is not specific because it rises in benign biliary obstruction and cholangitis, and it is useless in Lewis-antigen-negative patients who cannot make it, so it is best read as a trend in a high-risk patient. In a sclerosing cholangitis patient with a new dominant stricture and atypical but non-diagnostic ERCP brushings, the right next step is FISH on those brushings, where polysomy carries high specificity for cholangiocarcinoma. Intrahepatic cholangiocarcinoma larger than two centimeters is a contraindication to transplant, because occult micrometastatic disease drives poor post-transplant survival, so resection is the best chance. Perihilar transplant applies only to tumors at most three centimeters in radial diameter with no intrahepatic metastases or nodal involvement, following a fixed neoadjuvant chemoradiation, boost, maintenance, and staging-laparotomy sequence, and transperitoneal biopsy is contraindicated because it seeds the tract and disqualifies the patient. First-line systemic therapy is gemcitabine plus cisplatin plus durvalumab, and intrahepatic tumors earn a molecular workup because FGFR2 fusions are treated with pemigatinib or futibatinib and IDH1 mutations with ivosidenib. For the full chapter with MCQs, tables, and primary-guideline references, visit www.boardpearls.com. Questions or feedback: [email protected].

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“What did the council decide on item 42?” — answered with the speaker and the moment.

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