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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.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Ampullary adenoma or early T1 lesion | Endoscopic papillectomy with surveillance; surgery if invasive cancer or unfavourable features on pathology. | not mapped |
| Resectable carcinoma | Pancreatoduodenectomy with regional lymphadenectomy; biliary stenting first only if cholangitis or delayed surgery. | not mapped |
| Adjuvant | 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. | NCCN Category 2A |
| Metastatic | Subtype-directed chemotherapy; pembrolizumab for MSI-high; HER2-, BRAF- or NTRK-directed therapy where present; clinical trials. | not mapped |