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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.
Unresectable disease is treated with gemcitabine and cisplatin plus durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966), the biliary standards since 2022; FOLFOX is the second line after ABC-06, and liposomal irinotecan failed in NALIRICC. HER2-positive tumours respond to zanidatamab, which had a response rate of 41 percent in HERIZON-BTC-01 and gained accelerated FDA approval in 2024, and to trastuzumab deruxtecan; the phase 3 HERIZON-BTC-302 tests zanidatamab in the first line. Biliary stenting, endoscopic or percutaneous, and treatment of cholangitis are as important to survival as the anticancer drugs, since obstruction and infection are what usually end treatment.
Cancers of the bile ducts outside the liver, from the hilum where the ducts join to the lower duct near the pancreas; perihilar tumours are the commonest cholangiocarcinoma overall, and they present with jaundice, which brings both early symptoms and the risks of biliary obstruction.
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), Intrahepatic cholangiocarcinoma, 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.
Background: CA 19-9. Also on OnCo: Symptoms and red flags · Early detection roadmap.
MRI with cholangiography and CT for staging, endoscopic brushing or biopsy, and biliary drainage by stent or percutaneous route with antibiotics for cholangitis.
Bile duct resection with hemihepatectomy and caudate lobectomy after portal vein embolisation and drainage where needed, then six months of capecitabine (BILCAP).
Pancreaticoduodenectomy (Whipple) with lymphadenectomy, then adjuvant capecitabine.
Neoadjuvant chemoradiation followed by liver transplantation under the Mayo protocol in specialist centres.
Gemcitabine and cisplatin with durvalumab (TOPAZ-1) or pembrolizumab (KEYNOTE-966).
Zanidatamab (HERIZON-BTC-01) or trastuzumab deruxtecan; zanidatamab first line in HERIZON-BTC-302.
FOLFOX (ABC-06); pembrolizumab for microsatellite-unstable tumours, dabrafenib-trametinib for BRAF V600E.
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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.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Any new or worsening cough, breathlessness or fever. The label says to interrupt treatment for any suspected ILD and to permanently discontinue for grade 2 or higher.
Temperature of 38 C or higher, or feeling shivery and unwell even without a fever. Antibody-drug conjugates suppress the bone marrow, and several carry a boxed warning for severe neutropenia.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
See all on the product pages:CapecitabineDabrafenib + trametinibDurvalumabFOLFOX (5-FU, leucovorin, oxaliplatin)Gemcitabine + cisplatinPembrolizumabTrastuzumab deruxtecan·Printable cards in the navigator
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