Overview

Ampullary (AM-poo-la-ree) cancer is a rare cancer that forms in an area of your digestive system called the ampulla of Vater. The ampulla of Vater is located where your bile duct and pancreatic duct join and empty into your small intestine.

Ampullary cancer forms near many other parts of the digestive system, such as the liver, pancreas and small intestine. When ampullary cancer grows, it may affect these other organs.

Ampullary cancer treatment often involves extensive surgery to remove the cancer and a large margin of healthy tissue.

Ampullary cancer care at Mayo Clinic

Symptoms

Signs and symptoms of ampullary cancer may include:

  • Yellowing of the skin and eyes (jaundice)
  • Clay-colored stools
  • Abdominal pain
  • Fever
  • Bleeding from the rectum
  • Nausea
  • Vomiting
  • Weight loss

When to see a doctor

Make an appointment with your doctor if you have any persistent signs or symptoms that worry you.

Causes

It's not clear what causes ampullary cancer.

In general, cancer starts when cells develop changes (mutations) in their DNA. A cell's DNA contains the instructions that tell the cell what to do. The changes tell the cell to begin multiplying uncontrollably and to continue living when normal cells would die. The accumulating cells form a tumor that can invade and destroy normal body tissue.

Risk factors

Factors that can increase the risk of ampullary cancer include:

  • Your age. Ampullary cancer is more common in adults older than 70.
  • Being male. Males are slightly more likely to develop ampullary cancer than are females.
  • Inherited syndromes that increase cancer risk. Some gene mutations passed through generations of your family can increase your risk of ampullary cancer significantly. Only a small percentage of ampullary cancers are linked to inherited genes. The most common inherited syndromes that increase ampullary cancer risk are familial adenomatous polyposis and Lynch syndrome, which is also known as hereditary nonpolyposis colorectal cancer.

Ampullary cancer care at Mayo Clinic

Oct. 30, 2021

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  1. Feldman M, et al. Tumors of the bile ducts, gallbladder and ampulla. In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Saunders Elsevier; 2016. https://www.clinicalkey.com. Accessed June 13, 2019.
  2. Ryan DP, et al. Ampullary carcinoma: Treatment and prognosis. https://www.uptodate.com/contents/search. Accessed Sept. 8, 2019.
  3. Warner KJ. Allscripts EPSi. Mayo Clinic, Rochester, Minn. July 3, 2019.
  4. Martin JA. Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging. https://www.uptodate.com/contents/search. Accessed Sept. 8, 2019.