Disease Directory Ampullary Carcinoma
Oncology

Ampullary Carcinoma

Also known as: ampulla of Vater cancer, periampullary cancer, ampullary adenocarcinoma

Prevalence

0.2–0.5 in 100,000

Onset

Adult (median age 60s)

Type

Sporadic; FAP-associated in familial cases

About Ampullary Carcinoma

Ampullary carcinoma is a rare malignancy arising at the ampulla of Vater, the confluence of the common bile duct and pancreatic duct at the duodenal papilla, and is classified into intestinal and pancreatobiliary subtypes based on histological and immunohistochemical phenotype. The intestinal subtype shares molecular features with colorectal adenocarcinoma, including frequent KRAS and APC mutations, while the pancreatobiliary subtype resembles pancreatic ductal adenocarcinoma in biology and prognosis. Despite its rarity, ampullary carcinoma carries a relatively favourable prognosis among periampullary tumours when resected at an early stage, with five-year survival rates of 30–50% following pancreaticoduodenectomy (Whipple procedure).

Common Clinical Features

Obstructive jaundice: painless jaundice, dark urine, and pale stools from biliary obstruction at the ampulla Intermittent jaundice caused by tumour necrosis and temporary relief of obstruction (characteristic of ampullary location) Courvoisier sign: palpable, non-tender gallbladder from distension due to biliary obstruction Pancreatitis from pancreatic duct obstruction causing epigastric pain and elevated amylase/lipase Iron-deficiency anaemia or occult gastrointestinal bleeding from duodenal mucosal erosion Weight loss, anorexia, and fatigue Cholangitis: fever, rigors, and right upper quadrant pain from infected bile in cases with biliary stasis

Clinical Trial Eligibility Tips

What to know before applying to Ampullary Carcinoma trials.

Histological subtype (intestinal vs. pancreatobiliary) is an important stratification factor in most trials; request immunohistochemical panel including CK7, CK20, CDX2, and MUC markers to establish subtype.

MSI/MMR status and KRAS mutation status must be documented as they are eligibility and predictive biomarkers for immunotherapy and targeted therapy trials respectively.

Surgical resection status (R0 vs. R1 resection vs. unresectable) and biliary drainage adequacy (stent patency, bilirubin normalisation) are prerequisites for most systemic therapy trials — ensure bilirubin is within protocol-specified limits before screening.

Patient Resources

Patient Organization

Pancreatic Cancer Action Network

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Orphanet

European reference resource for rare diseases (ORPHA:67380)

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NORD

National Organization for Rare Disorders

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Find recruiting Ampullary Carcinoma trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Ampullary Carcinoma. Updated daily.

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