Overview
Carcinoma of the ampulla of Vater, also called ampullary cancer or ampullary carcinoma, is a rare cancer that forms at the ampulla of Vater — a small opening where the bile duct and pancreatic duct meet and empty into the small intestine (duodenum). Because of its location, this cancer can block the flow of bile and pancreatic juices, which often leads to noticeable symptoms relatively early compared to other cancers in the same area, such as pancreatic cancer. The most common early symptom is jaundice, which causes yellowing of the skin and eyes. Patients may also experience unexplained weight loss, abdominal pain, nausea, loss of appetite, pale or clay-colored stools, dark urine, and itching. Some patients develop pancreatitis or digestive problems because the tumor blocks normal drainage of digestive fluids. Treatment typically involves surgery, which offers the best chance for a cure when the cancer is caught early. The most common surgical procedure is the Whipple procedure (pancreaticoduodenectomy), which removes the head of the pancreas, the duodenum, part of the bile duct, and the gallbladder. Chemotherapy and sometimes radiation therapy may be used after surgery or for cancers that cannot be surgically removed. Compared to pancreatic cancer, ampullary cancer generally has a better prognosis because it tends to cause symptoms earlier, leading to diagnosis at an earlier stage. However, outcomes depend heavily on the stage at diagnosis and whether the cancer has spread to lymph nodes or other organs.
Also known as:
Key symptoms:
Yellowing of the skin and eyes (jaundice)Unexplained weight lossAbdominal or belly painNausea and vomitingLoss of appetitePale or clay-colored stoolsDark-colored urineItchy skinFatigue and weaknessBack painFever and chills (if bile duct becomes infected)Diarrhea or fatty stoolsNew-onset diabetes in some casesBloating or feeling full quickly
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsCui Xiaobing — NA
Yale University — PHASE2
Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA) — PHASE4
Minia University — PHASE3
Insel Gruppe AG, University Hospital Bern — NA
The Affiliated Nanjing Drum Tower Hospital of Nanjing University Medical School — PHASE2
Dartmouth-Hitchcock Medical Center — NA
Fondazione Poliambulanza Istituto Ospedaliero — NA
Affiliated Hospital of Guangdong Medical University — NA
Compass Therapeutics — PHASE2, PHASE3
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Carcinoma of the ampulla of Vater.
11 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Carcinoma of the ampulla of Vater.
Community
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Start the conversation →Latest news about Carcinoma of the ampulla of Vater
Disease timeline:
New recruiting trial: Efficacy and Safety of TPIAT for Resectable Adenocarcinoma of the Pancreas Region at High Risk of Postoperative Fistula
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Duodenal Polyposis Classification in FAP
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Individualized Dose Escalation of 5-FU for Gastrointestinal Cancer
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Neoadjuvant vs Upfront Surgery for Resectable Pancreatic Cancer and Periampullary Cancer
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Quebec Pancreas Cancer Study
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Trial Comparing Standard of Care Therapy With and Without Sequential Cytoreductive Intervention for Patients With Metastatic Foregut Adenocarcinoma and Undetectable Circulating Tumor-Deoxyribose Nucleic Acid (ctDNA) Levels
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: ToPanc Trial: Survival After Total Versus Partial Pancreaticoduodenectomy for Adenocarcinoma of the Pancreatic Head, Distal Cholangiocarcinoma, and Ampullary Cancer
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Pilot Comparing ctDNA IDV vs. SPV Sample in Pts Undergoing Biopsies for Hepatobiliary and Pancreatic Cancers
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: The ADAPTA Study: ADjuvant chemotherAPy After Curative Intent resecTion of Ampullary Cancer.
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
New recruiting trial: Preventing Postoperative Complications in Patients Undergoing High-risk Pancreatoduodenectomy With a Bundle Approach Including Hydrocortisone, Octreotide, and the Teres Ligament Patch (PANENCA)
A new clinical trial is recruiting patients for Carcinoma of the ampulla of Vater
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What stage is my ampullary cancer, and has it spread beyond the ampulla?,Am I a candidate for the Whipple surgery, and what are the risks and benefits?,Should my tumor be tested for microsatellite instability or other molecular markers that could guide treatment?,What chemotherapy regimen do you recommend after surgery, and what are the expected side effects?,Should I or my family members be tested for inherited conditions like familial adenomatous polyposis or Lynch syndrome?,What dietary changes will I need to make after surgery, and will I need pancreatic enzyme supplements?,Are there any clinical trials available for my type and stage of cancer?
Common questions about Carcinoma of the ampulla of Vater
What is Carcinoma of the ampulla of Vater?
Carcinoma of the ampulla of Vater, also called ampullary cancer or ampullary carcinoma, is a rare cancer that forms at the ampulla of Vater — a small opening where the bile duct and pancreatic duct meet and empty into the small intestine (duodenum). Because of its location, this cancer can block the flow of bile and pancreatic juices, which often leads to noticeable symptoms relatively early compared to other cancers in the same area, such as pancreatic cancer. The most common early symptom is jaundice, which causes yellowing of the skin and eyes. Patients may also experience unexplained weig
How is Carcinoma of the ampulla of Vater inherited?
Carcinoma of the ampulla of Vater follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Carcinoma of the ampulla of Vater typically begin?
Typical onset of Carcinoma of the ampulla of Vater is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Carcinoma of the ampulla of Vater?
Yes — 11 recruiting clinical trials are currently listed for Carcinoma of the ampulla of Vater on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Carcinoma of the ampulla of Vater?
23 specialists and care centers treating Carcinoma of the ampulla of Vater are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.