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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.
For advanced disease gemcitabine with cisplatin (ABC-02, 2010) was the standard for a decade until TOPAZ-1 (2022) added durvalumab and KEYNOTE-966 (2023) added pembrolizumab, each modestly improving survival and producing a tail of long-term responders. After chemotherapy, targeted drugs matched to the tumour's mutation are given: pemigatinib (FIGHT-202, response rate 36 percent) and futibatinib (FOENIX-CCA2, response rate 42 percent) for FGFR2 fusions, and ivosidenib for IDH1 mutations, which in ClarIDHy extended progression-free survival from 1.4 to 2.7 months and gave a survival benefit once crossover was accounted for. Acquired resistance to FGFR inhibitors through gatekeeper mutations is tracked with circulating tumour DNA, and next-generation FGFR2 inhibitors such as tinengotinib are in trials.
Bile duct cancer arising within the liver, the second commonest primary liver cancer after hepatocellular carcinoma and rising in incidence worldwide; it carries most of the targetable mutations in biliary cancer, with FGFR2 fusions in about one in eight and IDH1 mutations in about one in seven.
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
Same organ: Glucagonoma, VIPoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma, Adenosquamous carcinoma of the pancreas, Colloid (mucinous non-cystic) carcinoma of the pancreas, Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, Invasive carcinoma arising in an intraductal papillary mucinous neoplasm (IPMN-associated carcinoma), Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma), Solid pseudopapillary neoplasm of the pancreas
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Contrast MRI and CT, biopsy, and comprehensive genomic profiling including RNA-based fusion detection for every patient.
Hepatectomy with lymph node dissection followed by six months of adjuvant capecitabine (BILCAP).
Radioembolisation, stereotactic radiotherapy or hepatic artery infusion in specialised centres, usually with systemic therapy; liver transplantation in trials.
Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).
Pemigatinib (FIGHT-202) or futibatinib (FOENIX-CCA2); management of hyperphosphataemia and eye toxicity.
FOLFOX (ABC-06); trials.
Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Fever, cough or breathlessness, rapid weight gain or swelling, bone pain, low blood pressure or reduced urine; the labels say to start steroids and monitor at the first suspicion, and the syndrome has been fatal.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:CapecitabineDurvalumabFOLFOX (5-FU, leucovorin, oxaliplatin)FutibatinibGemcitabine + cisplatinIvosidenibPembrolizumabPemigatinib·Printable cards in the navigator
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