Ampullary cancer is a rare and aggressive type of carcinoma that forms in an area of your body called the ampulla of Vater. The ampulla of Vater is a small opening where your bile duct and pancreatic duct join. The most common symptom is jaundice, or yellowing, in your skin and eyes. Treatments include surgery, chemotherapy and radiation therapy.
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.
This type of cancer is rare. Ampullary cancer accounts for less than 1% of all cancers affecting the gastrointestinal (GI) tract.
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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:
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.
Factors that can increase your risk of developing ampullary cancer include:
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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:
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:
Ask your healthcare provider to explain what your cancer stage means for your treatment options.
Ampullary cancer treatment options include:
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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.
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.
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.
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.
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:
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.
Learning you have ampullary cancer can feel overwhelming. The answers you wish were most straightforward — like what your diagnosis means for your timeline and quality of life — may be the hardest to find answers to. This is because no matter what cancer statistics say, every cancer diagnosis is different.
Your healthcare provider is your best resource for explaining what to expect based on your situation. Don’t be afraid to ask hard questions. Your provider is there to answer honestly and help you choose your best options. It’s also a good idea to join support groups so you can connect with others who are dealing with a cancer diagnosis. And remember to lean on your loved ones. Finding the correct information, care and emotional support can help you manage your diagnosis.
Last reviewed on 07/08/2024.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy