Cancer Treatment Centers of America

Ampullary cancer: Know the signs of this rare disease

blog ampullary cancer

Earlier this year, ampullary cancer made the news when 1970s actress Karen Black succumbed to the disease. But many people who read the headline may have wondered, “What is ampullary cancer?” A rare gastrointestinal cancer, ampullary cancer develops in the ampulla of Vater, where the bile and pancreatic ducts meet and empty into the small intestine.

The most obvious symptom of ampullary cancer is jaundice, yellowing of the skin and the whites of the eyes. Jaundice is caused by an excess of bilirubin in the blood and body tissue. Bilirubin is a dark yellow-brown substance that develops when old red blood cells break down. Bilirubin is made in the liver, which excretes the substance into bile. 

Normally bile travels through the common bile duct into the intestines. It exits the body in stools. However, a tumor in the ampulla of Vater can block the common bile duct, preventing bile from reaching the intestines and causing a build-up of bilirubin. When this happens, the skin and eyes turn yellow and urine becomes a dark yellow or brown color.

According to the American Cancer Society (ACS), nearly everyone who has ampullary cancer has jaundice. In comparison, ACS says approximately 50 percent of people who are diagnosed with pancreatic cancer have jaundice. Because ampullary cancer causes such a noticeable symptom, it is often found at an earlier stage than pancreatic cancer.

Dr. Amer Alkhatib, a gastroenterologist at our hospital in Tulsa, estimates about a third of patients who are diagnosed with ampullary cancer also have mild gastrointestinal bleeding, which can lead to iron deficiency anemia. Less frequently, people who have the disease have severe gastrointestinal bleeding, which causes black stools or blood in stools. Other symptoms of ampullary cancer include nausea, vomiting, abdominal pain, weight loss and fever.

“Ampullary cancer can begin as an adenoma, a type of benign tumor which progresses over time to become cancer,” explains Dr. Alkhatib. “Colon cancer develops in a similar manner. It starts as an adenoma polyp in the colon which eventually becomes cancer.”

When doctors suspect their patients have ampullary cancer, they often use ultrasound to examine the bile duct for tumors. Yet Dr. Alkhatib says ultrasound is not very effective at detecting the disease. He believes an abdominal CT scan is more accurate at detecting ampullary cancer. A special endoscopy called an endoscopic retrograde cholangiopancreatography, or ERCP, can then be used to look closely at the ampulla of Vater and obtain biopsies to confirm the diagnosis.

Learn more about gastrointestinal cancers, including pancreatic, colorectalliver and stomach cancer.