Ampullary Cancer

What is ampullary cancer?

Ampullary cancer is a rare and aggressive (fast-growing) cancer that forms inside the ampulla of Vater. The ampulla of Vater is the opening where the ducts that carry digestive juices from your pancreas and liver meet. These juices combine in the ampulla of Vater before draining into the first part of your small intestine (duodenum).

As ampullary cancer forms so close to your liver, pancreas and other digestive system organs, it can easily spread to these areas. Treating ampullary cancer with surgery before it spreads offers the best outlook for the disease.

Another name for ampullary cancer is ampullary carcinoma.

How common is ampullary cancer?

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

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 your bile ducts. As a result, the backed-up bile (a yellowish fluid) flows into your bloodstream instead of your small intestine (where it’s supposed to go).

Other ampullary cancer symptoms include:

• Unexplained weight loss.
• Loss of appetite.
• Nausea and vomiting.
• Abdominal pain.
• Back pain.
• Itchy skin.
• Fever.
• Diarrhea.
• Anemia.
• Pancreatitis.
• Rectal bleeding.
• Fatty poops (may be greasy and clay-colored).

What causes ampullary cancer?

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

Experts continue to learn more about the specific types of mutations associated with ampullary cancer. This information helps them develop treatments that target the abnormalities and kill cancer cells. But this research is still in its early stages.

Risk factors

Factors that can increase your risk of developing ampullary cancer include:

• Being over the age of 70.
• Being assigned male at birth.
• Having inherited syndromes that increase your overall cancer risk, such as familial adenomatous polyposis (FAP), Lynch syndrome and Peutz-Jeghers syndrome.

How is ampullary cancer diagnosed?

Because the first sign of ampullary cancer is usually jaundice, yellowing of the skin is often the reason healthcare providers begin testing for the disease.

Tests to diagnose ampullary cancer include:

• Blood or urine tests to check for tumor markers.
• Imaging tests that use machines outside of your body to check for tumors inside of your body. Tests may include CT (computed tomography) scans, ultrasounds or magnetic resonance cholangiopancreatography (MRCP).
• Endoscopic tests that use a long, slender tube with a camera (endoscope) to access the tumor up close. Tests may include upper endoscopy and endoscopic retrograde cholangiopancreatography (ERCP). You’ll be sedated for these procedures, so you won’t feel any pain.
• A biopsy, which involves collecting a small tissue sample that a pathologist can test for cancer cells. Providers often collect the sample during an endoscopic test.

Ampullary cancer staging

The tests that allow healthcare providers to diagnose cancer also help with cancer staging. Staging gives information about how advanced the cancer is and what treatments will work best. Early-stage ampullary cancer responds better to treatment than cancer in advanced stages.

The stages of ampullary cancer are:

• Stage I (1): The tumor is only in the ampulla of Vater and potentially the muscular ring surrounding it (sphincter of Oddi). It may have spread to the inner or middle layers of your small intestine.
• Stage II (2): The tumor has spread to your pancreas or the outer layer of your small intestine.
• Stage III (3): The tumor has spread to your lymph nodes.
• Stage IV (4): The tumor has spread to other areas of your body (metastasis). When ampullary cancer metastasizes, it usually spreads to your liver, peritoneum (abdominal lining), lungs and other organs.

Ask your healthcare provider to explain what your cancer stage means for your treatment options.

How is ampullary cancer treated?

Ampullary cancer treatment options include:

• Whipple procedure: This surgical procedure is the most common ampullary cancer treatment. Providers remove the tumor and nearby areas that may also contain cancer cells, like your gallbladder, part of your bile duct and small intestine, and the head of your pancreas.
• Endoscopic surgery: Providers sometimes remove early-stage ampullary cancers during an endoscopy. During surgery, providers remove cancer cells using tools inside the endoscope.
• Chemotherapy: Your provider may recommend cancer-killing drugs after surgery to eliminate any remaining cancer cells. Chemotherapy drugs can also slow tumor growth even if they can’t get rid of a tumor completely.
• Combined chemotherapy and radiation therapy: Radiation therapy uses powerful beams of energy to destroy cancer cells. You may need this treatment alongside chemotherapy before surgery to shrink your tumor. Or you may need it after surgery to kill remaining cancer cells.
• Palliative care: These treatments focus on reducing uncomfortable symptoms. For example, your provider may place a stent (a small wire mesh tube) to open your bile duct, so bile won’t get backed up. The stent can help reduce jaundice and other symptoms.

Can I prevent ampullary cancer?

None of the risk factors associated with ampullary cancer are avoidable, so there isn’t anything you can do to prevent it.

If you have an inherited syndrome that increases your risk, your provider will recommend regular cancer screenings. You may not be able to prevent ampullary cancer. But early diagnosis increases your chances of getting treatments that can cure or manage it.

Is there a cure for ampullary cancer?

The only potential cure for ampullary cancer is surgery that removes it completely. Curative treatments usually only work with early-stage cancers. If there’s no way to remove the tumor completely, your healthcare team can work with you on treatments to slow tumor growth and reduce your symptoms.

In some cases, cancer goes into remission after surgery (no sign of cancer or symptoms) but then returns (recurrent cancer). This happens about half the time in ampullary cancer.

Your healthcare provider will continue to monitor you even if you’re healing well and cancer-free. You may need frequent follow-up visits in the first five years after surgery. The first five years are when cancer is most likely to return.

What is the survival rate of ampullary cancer?

Ampullary cancer is life-threatening, and survival rates decrease as the cancer stage increases. The five-year survival rate for people who receive the Whipple procedure ranges from 35% to 62%. This means that up to 62 out of 100 people who had the Whipple procedure for ampullary cancer are still alive after five years.

Keep in mind, though, that survival rates are only estimates. They can’t account for all the specific things about your diagnosis, including your overall health or how you’ll respond to treatment. To learn more about your outlook, talk to your healthcare provider.

When should I see my healthcare provider?

If you have ampullary cancer, call your healthcare provider anytime you notice new symptoms. Contact them immediately if you develop signs of an infection, like a fever.

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 tests will I need?
• What stage is my cancer?
• What are my treatment options?
• What side effects can I expect?
• How likely is remission?
• What resources do you recommend so that I can learn more about my diagnosis?
• What resources can help me connect with other people living with ampullary cancer?

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

The ampulla of Vater is centrally located among several digestive system organs. For this reason, experts call cancers that form near the ampulla of Vater periampullary cancers. “Peri” means about or around. For example, pancreatic cancer is a periampullary cancer.

The names are similar, but these cancers aren’t exactly the same. Providers may treat periampullary cancers (like pancreatic cancer) in much the same ways as ampullary cancer. Your provider will customize your treatment based on your cancer stage and individual needs.