Loading
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.
Intrahepatic cholangiocarcinoma starts in the small bile ducts inside the liver and usually appears as a liver mass rather than causing jaundice. It is the biliary cancer with the most drug targets: FGFR2 fusions treated with pemigatinib or futibatinib, IDH1 mutations with ivosidenib, and for everyone chemotherapy with the immunotherapy durvalumab or pembrolizumab.
Intrahepatic cholangiocarcinoma arises from the bile ducts beyond the second-order branches within the liver and presents as a mass, often found incidentally or with vague pain and weight loss, in contrast to the jaundice of extrahepatic tumours. Risk factors include cirrhosis, hepatitis B and C, primary sclerosing cholangitis, liver flukes in Thailand and neighbouring countries, and hepatolithiasis, but most cases in the West have none. Its genome differs from that of the rest of the biliary tree: FGFR2 fusions occur in about 10 to 15 percent, IDH1 mutations in about 15 percent, and BAP1 and ARID1A mutations are common, whereas KRAS and HER2 alterations are less frequent than in extrahepatic disease, so comprehensive sequencing at diagnosis is standard.
Resection is the only cure and is possible in a minority; hepatectomy with lymph node dissection is followed by six months of capecitabine after the BILCAP trial, whose per-protocol analysis showed longer survival. Recurrence is common. Liver transplantation for very early tumours in cirrhotic livers and after chemotherapy for locally advanced disease is under study, and for unresectable liver-confined disease radioembolisation, stereotactic radiotherapy and hepatic artery infusion chemotherapy are used in specialised centres.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis | Contrast MRI and CT, biopsy, and comprehensive genomic profiling including RNA-based fusion detection for every patient. | not mapped |
| Resectable | Hepatectomy with lymph node dissection followed by six months of adjuvant capecitabine (BILCAP). | not mapped |
| Unresectable, liver-confined | Radioembolisation, stereotactic radiotherapy or hepatic artery infusion in specialised centres, usually with systemic therapy; liver transplantation in trials. | not mapped |
| Advanced, first line | Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966). | not mapped |
| Advanced, FGFR2 fusion after chemotherapy | Pemigatinib (FIGHT-202) or futibatinib (FOENIX-CCA2); management of hyperphosphataemia and eye toxicity. | not mapped |
| Advanced, IDH1 mutation after chemotherapy | Ivosidenib (ClarIDHy). | not mapped |
| Second line without a target | FOLFOX (ABC-06); trials. | not mapped |