Ampullary cancer: 9 insights on this rare digestive system cancer

Ampullary cancer is a rare type of gastrointestinal cancer with symptoms so similar to pancreatic cancer that it is sometimes mistaken for the disease. The definitive way to confirm an ampullary cancer diagnosis is by removing a tissue sample from the tumor, then analyzing it in the lab.

To learn more about this uncommon disease that accounts for only 1% of all gastrointestinal cancers, we spoke with Michael Overman, M.D., who treats patients in MD Anderson’s Gastrointestinal Cancer Center.

What is ampullary cancer?

Ampullary cancer is an aggressive cancer that forms in an area of the digestive tract called the ampulla of Vater.

Where is the ampulla of Vater, and what is its function?

The ampulla of Vater is a tiny opening in the small intestine. The pancreatic duct and the liver’s bile duct join together at the ampulla of Vater, where they empty their bile and digestive enzymes through the opening and into the small intestine. This helps the body digest food.

The ampulla of Vater’s name comes from “ampulla,” which means “channel” in anatomy, and “Vater” – the last name of Abraham Vater, the German anatomist who discovered the structure.

How does ampullary cancer develop?

Ampullary cancer occurs when cells in the ampulla of Vater begin to grow out of control and form a tumor. If cancer remains undetected and untreated, it can spread to the nearby pancreas, liver, bile duct and small intestine. The gastrointestinal system is very compact, so cancer that begins in the ampulla of Vater doesn’t have far to travel.

When ampullary cancer spreads to another organ, the pancreas, for example, it is still called “ampullary cancer that has spread to the pancreas,” not pancreatic cancer.

What are common ampullary cancer symptoms?

The most common ampullary cancer symptom is jaundice – yellowing of the skin and eyes. This happens because a tumor in the ampulla of Vater blocks the bile duct from emptying its contents into the small intestine. The bile has nowhere to go, so it backs up into the bloodstream and causes jaundice.

The good news is that jaundice leads people to visit their doctors. And because jaundice is usually the first sign of ampullary cancer, the disease is often caught early. This results in a better prognosis for ampullary cancer, compared to pancreatic cancer and other types of gastrointestinal cancers.

Besides jaundice, other ampullary cancer symptoms include:

• weight loss
• nausea or vomiting
• abdominal and back pain
• severe itching
• diarrhea
• digestive tract bleeding
• pancreatitis (inflammation of the pancreas)
• stools that appear pale and greasy

What causes ampullary cancer?

It’s not clear what causes ampullary cancer. But we know that people with inherited conditions like familial adenomatous polyposis (FAP) or Lynch syndrome that cause polyps to grow in the digestive system are more likely to develop the disease.

How is ampullary cancer diagnosed?

A tumor tissue biopsy can provide a definitive diagnosis. Other tests can help doctors stage the cancer and develop a treatment plan. These include:

• CT scans, MRIs or ultrasounds to identify the size and location of tumors
• Endoscopy, to remove small tissue samples from the ampulla of Vater for biopsy
• Endoscopic retrograde cholangiopancreatography (ERCP), to determine if the pancreatic or bile ducts are blocked

How is ampullary cancer treated?

A surgery called the Whipple procedure is the most common treatment for ampullary cancer. The surgeon removes the tumor in the ampulla of Vater, as well as several adjoining areas where cancer may have spread. These include the head of the pancreas, part of the small intestine, the gallbladder, nearby lymph nodes, and, in some cases, the lower half of the stomach.

Some patients undergo chemotherapy and radiation therapy after surgery to kill any remaining cancer cells.

Patients who are not healthy enough to undergo a Whipple procedure may be treated with chemotherapy, radiation therapy, or laser surgery that uses special light beams instead of a scalpel to cut tissue.

What is the prognosis for someone diagnosed with ampullary cancer?

The five-year survival rate for people treated with the Whipple procedure ranges from 20% to 75%, based on how far the cancer has spread. This means that up to 75 out of 100 people who had the Whipple procedure for ampullary cancer are still alive after five years.

The cell types that make up the tumor also impact prognosis. Ampullary tumors include intestinal, pancreatic or biliary cells, or a mix of those types. Tumors composed mainly of intestinal cells have the best prognosis.  

What is your best advice for someone diagnosed with ampullary cancer?

Many fine cancer specialists work in private practice and in smaller hospitals and clinics, but because ampullary cancer is so rare, they may not have encountered patients with your diagnosis. Trust your care to a comprehensive cancer center like MD Anderson, where everyone on your treatment team – from pathologists to radiologists to oncologists and surgeons – has experience with ampullary cancer.

Request an appointment at MD Anderson online or by calling 1-877-632-6789.