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Ampullary cancer, a biliary tract cancer, starts where the bile and pancreatic ducts empty into the small bowel. Because it blocks bile flow early it is often caught while still removable, and the Whipple operation cures a good share of patients. Tumours come in two flavours, intestinal-like and pancreas-like, and chemotherapy is increasingly chosen by which one the pathologist sees.
Ampullary adenocarcinoma arises from the ampulla of Vater, the papilla where the common bile duct and main pancreatic duct open into the duodenum. It is grouped with periampullary cancers (distal cholangiocarcinoma, duodenal adenocarcinoma, pancreatic head cancer) at surgery but behaves better than pancreatic cancer because it obstructs the bile duct and is diagnosed early. Two histomolecular subtypes matter: intestinal-type tumours resemble colorectal cancer (CDX2, MUC2; APC and KRAS mutations) and pancreatobiliary-type tumours resemble pancreatic cancer (MUC1, CK7; KRAS, TP53, SMAD4), with the latter behaving more aggressively. Familial adenomatous polyposis carries a large relative risk of ampullary adenoma and carcinoma, and endoscopic surveillance of the duodenum is part of FAP care.
Curative treatment is pancreatoduodenectomy (Whipple); small adenomas and some early T1 lesions can be removed endoscopically by papillectomy. Adjuvant chemotherapy is extrapolated: ESPAC-3 periampullary (JAMA 2012) showed a survival advantage for adjuvant gemcitabine or fluorouracil in multivariable analysis, and practice now leans on subtype, with oxaliplatin-fluoropyrimidine (FOLFOX or CAPOX) for intestinal-type and gemcitabine-based or modified FOLFIRINOX regimens for pancreatobiliary-type tumours. For metastatic disease the same logic applies, and tumour-agnostic biomarkers (MSI-high, HER2, NTRK, BRAF) should be tested because they are found more often than in pancreatic cancer.
The active questions are prospective subtype-directed adjuvant trials, ctDNA to guide adjuvant decisions, and neoadjuvant therapy for node-positive disease.
Rare: well under one case per 100,000 per year, but it makes up a disproportionate share of resectable pancreatoduodenectomies because it obstructs the bile duct early and presents with jaundice.
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, 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, 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.
Background: CA 19-9. Also on OnCo: Symptoms and red flags · Early detection roadmap.
Endoscopic papillectomy with surveillance; surgery if invasive cancer or unfavourable features on pathology.
Pancreatoduodenectomy with regional lymphadenectomy; biliary stenting first only if cholangitis or delayed surgery.
Six months of chemotherapy chosen by subtype: oxaliplatin-fluoropyrimidine (FOLFOX or CAPOX) for intestinal-type; gemcitabine-based or modified FOLFIRINOX for pancreatobiliary-type; evidence is from ESPAC-3 periampullary and retrospective series.
Subtype-directed chemotherapy; pembrolizumab for MSI-high; HER2-, BRAF- or NTRK-directed therapy where present; clinical trials.
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Japanese surgeons operate on gallbladder cancer far more often than UK surgeons and their guideline addresses prevention (pancreaticobiliary maljunction, polyps) as a clinical topic, which Western guidelines do not.
The highest gallbladder HER2 rate in the literature comes from whole resected tumours scored generously; biopsy-based trial screening finds fewer. Heterogeneity is the practical warning for pathologists and for why HER2-directed drugs do not work in every positive tumour.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Ampullary cancer" OR ABSTRACT:"Ampullary cancer" OR TITLE:"ampulla of Vater" OR ABSTRACT:"ampulla of Vater" OR TITLE:"Ampulla of Vater carcinoma" OR ABSTRACT:"Ampulla of Vater carcinoma" OR TITLE:"Periampullary cancer" OR ABSTRACT:"Periampullary cancer" OR TITLE:"Ampullary adenocarcinoma" OR ABSTRACT:"Ampullary adenocarcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Ampullary cancer (ampulla of Vater), not a curated reading list.
First successful local resection of a periampullary tumour.
Allen Whipple reports the operation that became the standard for periampullary cancer.
Kimura and colleagues link histological type to prognosis.
Adjuvant chemotherapy after resection of periampullary cancer; survival benefit in adjusted analysis.
Subtype-directed treatment recommendations formalised.
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.
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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.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
The three main regimens share low blood counts, tiredness, sickness and sore mouth; FOLFIRINOX and NALIRIFOX add irinotecan diarrhoea and oxaliplatin's cold-triggered tingling and rare throat spasm, gemcitabine with nab-paclitaxel adds hair loss and neuropathy, and every regimen comes with the same temperature rule for ringing the 24-hour line.
See all on the product pages:CAPOX (capecitabine, oxaliplatin)Dabrafenib + trametinibFOLFIRINOX / mFOLFIRINOXFOLFOX (5-FU, leucovorin, oxaliplatin)GemcitabineGemcitabine + nab-paclitaxelPembrolizumab·Printable cards in the navigator
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