Ampullary Cancer

What is ampullary cancer?

Ampullary cancer forms in the ampulla of Vater, an opening that enters the duodenum (the first portion of your small intestine). The ampulla of Vater is located near other organs in your digestive system, including your liver and pancreas.

How common is ampullary cancer?

This type of cancer is fairly rare. Ampullary cancer accounts for less than 1% of all cancers affecting the gastrointestinal tract.

Who does ampullary cancer affect?

Ampullary cancer is most common in people over the age of 70. Males are more likely than females to develop the condition. Statistically, 0.74 out of every 100,000 males will develop ampullary cancer while 0.48 out of every 100,000 females will be diagnosed with the condition.

How aggressive is ampullary cancer?

Ampullary cancers grow fast and are more likely to spread. Generally, the more advanced the tumor, the faster it will grow.

Is ampullary cancer the same as pancreatic cancer?

No. However, ampullary cancer develops near the pancreatic duct and is treated in much the same way as pancreatic cancer.

Ampullary cancer vs periampullary cancer: What’s the difference?

Ampullary cancers form in the ampulla of Vater, while periampullary cancers originate in the bile duct, pancreas or small intestine (which is located close to the ampulla of Vater).

What are the symptoms of ampullary cancer?

People with ampullary cancer usually develop jaundice (yellowing of the eyes and skin). This is because the tumor can block the bile duct. As a result, bile is unable to move into the intestines and goes into the blood instead. Other ampullary cancer symptoms include:

• Weight loss.
• Loss of appetite.
• Nausea and vomiting.
• Abdominal pain.
• Back pain.
• Itchy skin.
• Fever.
• Diarrhea.
• Anemia.
• Pancreatitis.
• Clay-colored stools (poop).
• Rectal bleeding.

What causes ampullary cancer?

Experts aren’t sure what causes ampullary cancer. However, we know that cancer begins when cells develop mutations (changes) in their DNA. These abnormal cells start to multiply and grow out of control.

There are certain factors that can increase your risk of developing ampullary cancer. These risk factors include:

• Being over the age of 70.
• Being male.
• Having inherited syndromes that may increase your cancer risk, such as Lynch syndrome or familial adenomatous polyposis (FAP).

How does ampullary cancer spread?

When ampullary cancer metastasizes, it usually spreads to the liver, lymph nodes, lungs, peritoneum (abdominal lining) and other organs. To better understand the process of metastasis, the stages of ampullary cancer are listed below:

• Stage 1: The cancer is only in the inner layer of your small intestine, and has not spread to nearby areas.
• Stage 2: The tumor has spread to the middle layer of your small intestine. The cancer may have also spread to the sphincter of Oddi — a muscular ring around the ampulla of Vater.
• Stage 3: The cancer has spread to the outer wall of your small intestine.
• Stage 4: The cancer has spread to other areas of your body. This may include lymph nodes or organs such as the liver or pancreas.

How is ampullary cancer diagnosed?

Because the first sign of ampullary cancer is usually jaundice, yellowing of the skin is often the reason that many healthcare providers begin testing for the disease. Tests used to diagnose ampullary cancer include:

• Blood or urine tests to check for cancer markers.
• Imaging tests to look for tumors, which may include CT (computed tomography) scans, ultrasounds or magnetic resonance imaging (MRI).
• Upper endoscopy, which involves using a tiny camera to check for cancer inside of your body.
• ERCP (endoscopic retrograde cholangiopancreatography), which can tell your provider if your bile duct is blocked.
• A biopsy, which involves collecting a small sample of cells for analysis.

How is ampullary cancer treated?

There are a few different options for treating ampullary cancer. Treatment recommendations will depend on the location, size and stage of your cancer and your overall health and healing capacity. Ampullary cancer treatment options include:

• Endoscopic surgery. Early stage ampullary cancers can sometimes be removed using this option. During endoscopic surgery, your surgeon removes cancer cells using tools passed through an endoscope (a small tube commonly used in minimally invasive procedures).
• Whipple procedure. This surgical procedure — also called a pancreaticoduodenectomy — is used to remove your gallbladder, part of your bile duct, a portion of your small intestine and the head of your pancreas.
• Chemotherapy. This approach uses cancer-killing drugs to target ampullary cancer cells. Chemotherapy is often used after surgery to eliminate any remaining cancer cells. But it may also be used to slow the growth of the tumor.
• Combined chemotherapy and radiation therapy. Chemotherapy is often used with radiation therapy in the treatment of ampullary cancers. Radiation therapy uses powerful beams of energy to target cancer cells. This combination may be used before surgery to shrink your tumor, or after surgery to kill remaining cancer cells.
• Treatments focused on reducing uncomfortable symptoms. When other treatments don’t help, your provider may recommend placing a stent (a small wire mesh tube) in your bile duct to reduce jaundice and other symptoms.

What are the complications of ampullary cancer treatment?

As with any procedure, complications are possible following ampullary cancer treatments. These complications can vary from person to person depending on which treatment you receive. People who undergo surgery for ampullary cancer may experience:

• Wound infection.
• Blood clots.
• Delayed gastric emptying.
• Pancreatic anastomotic leak, a condition in which pancreatic fluid leaks due to disruption of the pancreatic ducts.

People who receive chemotherapy or radiation therapy for ampullary cancer may experience a number of side effects, including fatigue, hair loss, nausea, diarrhea, constipation, infertility and flu-like symptoms.

If you’ve recently undergone treatment for ampullary cancer, be sure to attend all scheduled follow-ups. These appointments allow your healthcare provider to monitor your health and address any complications before they worsen.

Can I prevent ampullary cancer?

Because there are no avoidable risk factors associated with ampullary cancer, there is currently no known way to prevent the condition.

Can ampullary cancer be cured?

The only potential cure for ampullary cancer is complete removal of the tumor. This treatment is most likely to be successful when the cancer is still in the earliest stage. However, if complete removal of the tumor isn’t possible, your healthcare team will help you find ways to manage your condition.

What is the survival rate of ampullary cancer?

Ampullary cancer is considered a life-limiting disease and survival rates decrease as the stage of disease increases. However, the five-year survival rate for people who undergo the Whipple procedure ranges from 20% to 61%. This means that up to 61 out of 100 people who had the Whipple procedure for ampullary cancer are still alive after five years. It’s important to note that survival rates are only estimates. They can’t tell you how you’ll respond to treatment or how long you’ll live. To learn more about survival rates for ampullary cancer, talk to your healthcare provider.

Can ampullary cancer come back?

Yes, it’s possible. Following surgical resection, there is still a 33% to 60% chance for recurrence.

When should I see my healthcare provider?

If you’ve been diagnosed with ampullary cancer, call your healthcare provider anytime you notice new symptoms or when you develop fever or other signs of infection.

What questions should I ask my healthcare provider?

Learning all you can about your diagnosis is essential for making well-informed decisions about your health. Here are some questions to consider asking your healthcare provider:

• What do my test results mean?
• Has my cancer spread beyond the ampulla of Vater?
• Will I need to undergo any other tests?
• What are my treatment options?
• What side effects can I expect?
• How likely is remission?
• How long do I have to make a decision about my treatment?
• What resources do you recommend so that I can learn more about my diagnosis?

A note from Cleveland Clinic

An ampullary cancer diagnosis can be overwhelming. You may feel sadness, frustration, worry or anger. Consider joining a support group, which will put you in contact with others who are going through the same things. Be frank and honest with your healthcare provider, and talk with them about any emotions you’re experiencing. They can find ways to help as you navigate this difficult time. Finding the right resources can give you the information you need to make the best decisions for your health.