What is ampullary cancer?

Ampullary cancer is a rare type of cancer. It occurs when cancer starts in the part of the body called the ampulla of Vater. The ampulla of Vater is a small opening where the pancreatic and bile ducts (from the liver) connect to the first part of the small intestine (the duodenum). These ducts release their secretions into the intestines.

Ampullary cancer may also be called ampulla of Vater cancer.

What causes ampullary cancer?

Experts aren’t sure what causes ampullary cancer. It occurs when cells in the body change and grow out of control. These abnormal cells may grow to form a lump or mass called a tumor. If they are in the body long enough, they can grow into (invade) nearby areas and spread to other parts of the body (metastasis).

Who is at risk for ampullary cancer?

A risk factor is anything that may increase your chance of having a disease. The exact cause of someone’s cancer may not be known. But risk factors may make it more likely for a person to have cancer. Some risk factors may not be in your control, but others may be things you can change. There are no known clear risk factors for ampullary cancer.

You may have a possible increased risk for this cancer if you:

• Have certain inherited health problems that cause growths (polyps) in the digestive system

• Have inflammatory bowel disease

• Are an older adult

Talk with your healthcare provider about any potential risk factors for ampullary cancer and what you can do about them.

What are the symptoms of ampullary cancer?

The most common symptom of this cancer is jaundice. This is yellowing of the skin and eyes. It happens because the tumor in the ampulla of Vater blocks the bile duct. If bile flow is blocked from going into the intestines, it goes into the blood and causes yellowing of the skin and eyes.

Other symptoms of the cancer are:

• Loss of appetite

• Weight loss

• Belly pain

• Back pain

• Itchy skin

• Stomach upset and vomiting

• Diarrhea

• Fever

• Digestive tract bleeding

• Low red blood cell counts (anemia)

• Inflammation of the pancreas (pancreatitis)

• Pale, greasy stools

• Fatigue

Many of these symptoms may be caused by other health problems. So it’s important to see your healthcare provider if you have these symptoms. Your provider will do exams and specific testing to find out if you have cancer.

How is ampullary cancer diagnosed?

Jaundice is the most common symptom of this cancer. It’s often what leads people to see a healthcare provider. Your provider will ask you about your health history, symptoms, risk factors, and family history of disease. They will do a physical exam. 

You may also have one or more of these tests:

• Blood and urine tests. These are used to look for anemia, bilirubin levels, and other changes that may be signs of this cancer.

• Imaging tests. These can be used to look for a tumor inside the ampulla of Vater. They may be ultrasounds or CT or MRI scans.

• Endoscopy. Healthcare providers may use an endoscope to look at the ampulla. An endoscope is a long, thin tube. It has a tiny video camera on the end. It’s threaded down the mouth, through the esophagus and stomach, and into the duodenum to the ampulla. It can also be used to remove small tissue samples that will be examined in a lab to find out if cancer cells are present.

• Endoscopic retrograde cholangiopancreatography (ERCP). This test might be used to look at the pancreatic and bile ducts to see if they are blocked.

It can be hard to tell the difference between pancreatic cancer and ampullary cancer. A biopsy is the only way to confirm this cancer. Small tissue samples are removed then examined under a microscope in a lab to find out the type of cancer cells present.

After a diagnosis of ampullary cancer, you’ll likely need more tests. These help your healthcare providers learn more about the cancer. They can help find out the stage of cancer. The stage is how much and how far the cancer has spread (metastasized) in your body. It’s one of the most important things to know when deciding how to treat the cancer.  

Once your cancer is staged, your healthcare provider will talk with you about what the stage means for your treatment. Ask your provider to explain the stage of your cancer to you in a way you can understand.

How is ampullary cancer treated? 

Your treatment choices depend on the type of ampullary cancer you have, test results, if the cancer can be removed with surgery, and the size and stage of the cancer. The goal of treatment may be to cure you, control the cancer, or to help ease problems caused by the cancer. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be.

Types of treatment for cancer are either local or systemic:

• Local treatments. These remove, destroy, or control cancer cells in one certain area. Surgery and radiation are local treatments.

• Systemic treatments. These are used to destroy or control cancer cells that may have traveled all over your body. When taken by pill or injection, chemotherapy is a systemic treatment.

You may have just one type of treatment or a combination of treatments.

The main treatment for ampullary cancer is surgery to remove the tumor. The Whipple procedure (also called a pancreaticoduodenectomy) is used. This is a major surgery where your surgeon removes the tumor in the affected part of the ampulla of Vater. Nearby tissues are often removed as well. These include the head of the pancreas, the lower half of the stomach, the duodenum, gallbladder, and lymph nodes.

Some people can’t have a Whipple procedure. In these cases, a less complex surgery or another procedure (such as using a laser to kill the tumor) may be done. It’s not clear if these methods can cure ampullary cancer. Some healthcare providers may also advise other treatments after surgery, such as chemotherapy or radiation therapy.

Talk with your healthcare providers about your treatment options. Make a list of questions. Think about the benefits and possible side effects of each option. Discuss your questions and concerns with your healthcare provider before making a decision.

When should I call my healthcare provider?

Call your healthcare provider or get medical care right away if you have:

• New symptoms or symptoms that get worse

• Signs of an infection, such as a fever

• Side effects of treatment that affect your daily function or don’t get better with treatment

Ask your healthcare provider what symptoms to watch for and when to call. Know how to get help after office hours and on weekends and holidays.

Key points about ampullary cancer

• Ampullary cancer forms in a body part called the ampulla of Vater. This is a small opening that connects the bile and pancreatic ducts to the duodenum.

• It’s a rare form of cancer.

• People with certain inherited conditions that cause growths (polyps) in the digestive system may be more at risk for this type of cancer.

• The most common symptom is jaundice or yellowing of the skin and eyes. It’s caused by bile going into the blood instead of into the duodenum.

• The main treatment is a type of surgery called the Whipple procedure. It removes the tumor and nearby tissues.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions.