Ampullary Cancer: The Basics

Named after the scientist who discovered it, the ampulla of Vater is found at the start of your small intestine (the duodenum). It is a hollow, empty, button-like bump on the inside wall of your duodenum.

The ampulla of Vater stores digestive juices until they are needed to digest (break down) food. Bile is a type of digestive juice made by your liver and gallbladder that helps you digest fat. Bile travels through the bile duct and meets at the ampulla of Vater with enzymes (proteins) from the pancreatic duct. The bile and enzymes then empty into your small intestine to break down food. There is a sphincter (a tight ring that opens and closes as needed) around the ampulla of Vater that only lets out digestive juices when there is food to digest.

When there are cancer cells in the ampulla of Vater, it is called ampullary cancer. As the cancer cells grow, a tumor forms. Tumors in this area affect the flow of digestive juices and how you digest food.

Risks

Risk factors for ampullary cancer are:

• Having a genetic condition, such as Lynch syndrome, Peutz-Jeghers syndrome, or familial adenomatous polyposis (FAP).
• Being male.
• Being over the age of 70.

Signs of Ampullary Cancer

Ampullary cancer tends to cause early signs and symptoms because it affects your digestion. Some signs may be:

• Jaundice (yellowing of your skin and eyes), caused by the tumor blocking the bile duct.
• Losing weight without trying.
• Diarrhea.
• Fatty, greasy, or clay-colored stool.
• Bleeding.
• Not feeling hungry.
• Nausea and/or vomiting.
• Belly pain.
• Fever.
• Swelling and inflamed pancreas (pancreatitis).

Diagnosing Ampullary Cancer

When your provider thinks you may have ampullary cancer, you will have a full review of your health and your body. Tests may be ordered, such as:

• CT.
• Ultrasound or endoscopic ultrasound.
• Endoscopic Retrograde Cholangiopancreatography (ERCP): A scope is passed through your mouth, esophagus, and stomach, and it is placed into the first part of your small intestine (duodenum). Here, a smaller tube is passed through the bile ducts. An X-ray is taken after dye is put in to look for any blockages. A stent may be placed to keep the duct open. A biopsy may also be taken.
• Magnetic Resonance Cholangiopancreatography (MRCP): During an MRCP, the liver, gallbladder, bile ducts, pancreas, and pancreatic duct are looked at using a certain type of MRI. A biopsy may also be taken.
• Blood and urine tests to check for tumor markers.

Staging Ampullary Cancer

Ampullary cancer is "staged." Staging helps guide your treatment. This stage is based on:

• Where and how big your tumor is.
• If there are cancer cells in your lymph nodes.
• If there are cancer cells in other parts of your body.

Stages range from stage I (1, smallest tumors) to stage IV (4, tumors that have spread to other parts of the body, called metastatic cancer). The stage of ampullary cancer will guide your treatment plan.

Treating Ampullary Cancer

Treatment for ampullary cancer can include:

• Whipple procedure: Surgery done to remove the head (and sometimes the body) of the pancreas, the gallbladder, bile duct, and part of the stomach (pylorus) and small intestine (duodenum). Nearby lymph nodes are also removed.
• Stent placement: A tube, called a stent, is placed into the bile duct to keep it open and working.
• Endoscopic surgery: If the tumor is small enough, the surgeon may be able to use small incisions (surgical cuts) and special tools to remove the tumor.
• Radiation is the use of high-energy X-rays to kill cancer cells. It is often used with chemotherapy.
• Chemotherapy uses medications to kill cancer cells that have spread to other places in the body. It can be used alone or with radiation.
• Targeted Therapy uses medications that target something specific in the cancer cells. Your tumor may be tested for certain tumor markers.
• Immunotherapy uses medications that help your immune system attack cancer cells. These medications are often used when your cancer spreads to other parts of your body, or if the ampullary cancer comes back (recurrence).

This article is a basic guide to ampullary cancer. Talk with your provider about your diagnosis, stage, and treatment options.