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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Extrahepatic cholangiocarcinoma blocks the main bile ducts outside the liver and shows itself as jaundice. Perihilar tumours need part of the liver removed with the duct and distal tumours a Whipple operation; where surgery is impossible, stenting relieves the jaundice and chemotherapy with immunotherapy follows, with HER2-directed antibodies for the one in six tumours that carry that target.
Extrahepatic cholangiocarcinoma is divided at the cystic duct into perihilar tumours, described by Klatskin in 1965 and classified by Bismuth and Corlette according to how far they extend into the right and left hepatic ducts, and distal tumours of the common bile duct. Both present with painless jaundice, pale stools, dark urine and itching, often with cholangitis, and CA 19-9 is raised but unreliable in the presence of obstruction. Primary sclerosing cholangitis, choledochal cysts and liver flukes are risk factors. The genome differs from intrahepatic disease: KRAS and TP53 mutations dominate, HER2 amplification occurs in about one in six, and FGFR2 fusions and IDH1 mutations are rare.
Surgery is the only cure and is among the most demanding in abdominal oncology: perihilar tumours require resection of the bile duct with the ipsilateral hemiliver and caudate lobe, often after portal vein embolisation to grow the remnant and biliary drainage to reverse jaundice, while distal tumours are removed by pancreaticoduodenectomy. Clear margins are achieved in perhaps half of cases. Adjuvant capecitabine for six months follows BILCAP. For unresectable perihilar tumours in primary sclerosing cholangitis or under 3 cm, the Mayo protocol of chemoradiation followed by liver transplantation achieves long-term survival in selected patients and is offered in a few centres.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis and jaundice | MRI with cholangiography and CT for staging, endoscopic brushing or biopsy, and biliary drainage by stent or percutaneous route with antibiotics for cholangitis. | not mapped |
| Resectable perihilar | Bile duct resection with hemihepatectomy and caudate lobectomy after portal vein embolisation and drainage where needed, then six months of capecitabine (BILCAP). | not mapped |
| Resectable distal | Pancreaticoduodenectomy (Whipple) with lymphadenectomy, then adjuvant capecitabine. | not mapped |
| Unresectable perihilar, selected | Neoadjuvant chemoradiation followed by liver transplantation under the Mayo protocol in specialist centres. | not mapped |
| Advanced, first line | Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966). | not mapped |
| Advanced, HER2-positive after chemotherapy | Zanidatamab (HERIZON-BTC-01) or trastuzumab deruxtecan; zanidatamab first line in HERIZON-BTC-302. | not mapped |
| Second line without a target | FOLFOX (ABC-06); pembrolizumab for microsatellite-unstable tumours, dabrafenib-trametinib for BRAF V600E. | not mapped |