About Cancer
Cancer is when abnormal cells start to divide and grow in an uncontrolled way. The cells can grow into surrounding tissues or organs and may spread to other areas of the body.
Ampullary cancer is when abnormal cells in the ampulla divide in an uncontrolled way. It is also called ampulla of Vater cancer.
Find out more about cancer and how it starts
When you eat, your releases a juice called bile into your common bile duct. Bile breaks down fat in the food you've eaten. This makes it easier for your body to use the food.
The bile flows down your common bile duct and joins your pancreatic duct. This releases a juice from your pancreas which also breaks down food.
The bile and pancreatic juice flow through the ampulla into the duodenum.
The ampulla is lined by a layer of tissue called epithelial tissue. There are different types of epithelial tissue cell. Most cancers of the ampulla start in epithelial cells that keep the tissue moist. Doctors call these cancers adenocarcinomas.
Epithelial cells are different in different parts of the body. In the ampulla, they can be similar to the cells that line the duodenum. Or they might be like the cells lining the pancreas and common bile duct. Sometimes the cells might be a mix of both.
Knowing which type of cell the cancer started in, may help your doctor decide on the best treatment for you.
Very rarely cancer can start in neuroendocrine cells in the ampulla. These cells control how our bodies work. Cancer that starts in neuroendocrine cells in the ampulla is different to ampullary cancer.
Find out more about neuroendocrine cancer
Ampullary cancer is very rare. In the UK, around 550 people are diagnosed with ampullary cancer each year.
Anything that can increase your risk of getting a disease is called a risk factor. Different cancers have different risk factors. Having one or more risk factor doesn't mean you will definitely get that cancer.
Doctors don’t know what causes ampullary cancer, but they think there may be a link with some health conditions caused by inherited gene changes. This includes (FAP). There may also be a link with . But the evidence for this is less clear.
Ampullary cancer can cause symptoms such as:
yellowing of the skin and whites of your eyes (jaundice)
blood in your poo
blood in your sick
inflammation of the pancreas (pancreatitis)
These symptoms can also be caused by many other conditions. Most of them are much more common than ampullary cancer.
If a cancer stops bile flowing into the duodenum, it goes into your bloodstream and tissues instead. This makes the whites of your eyes look yellow. Your skin may also appear yellow. This may be less noticeable on black or brown skin. The yellow colour is caused by the pigments in the bile.
Jaundice can also make you have:
light coloured poo
dark coloured pee
itchy skin
a high temperature (fever) - this may make you feel cold and shivery
This might be bright red, or it might be almost black if the blood has been in your stomach for a while.
This might be bright red or it may look dark brown, like used coffee grounds.
This can cause you to have a fever, feel or be sick and have pain in your upper tummy (abdomen). The pain may go around to your back.
pain in your abdomen not caused by pancreatitis
loose poo (diarrhoea)
unexplained weight loss
You should see a doctor if you have symptoms that could be due to ampullary cancer. They will examine you. And you normally have blood tests to check your general health and how well your liver is working.
They may refer you to a specialist doctor for further tests to find out the cause of your symptoms. These tests might include:
an scan of your abdomen
a of your chest, abdomen and the area between your hips (pelvis)
an to look at your food pipe (oesophagus), stomach and duodenum - this is also called a gastroscopy
an endoscopic ultrasound (EUS) - this combines an ultrasound scan and endoscopy
an endoscopy to look inside your pancreas and bile ducts - doctors call this endoscopic retrograde cholangio pancreatography (ERCP)
If your doctor finds an abnormal area during an endoscopy procedure, they take a sample from it. This is called a biopsy. A specialist doctor called a pathologist looks at the biopsy under a microscope. This is to see if it contains cancer cells.
Occasionally your doctor may want you to have other tests. They will explain what the tests are for and how to prepare for them.
Find out more about the tests for ampullary cancer
Your doctor considers many factors when deciding which treatment is suitable for you. This includes:
the size of the cancer and whether it has spread to other parts of your body (the stage)
if you can have surgery to remove the cancer
if the cancer has any gene changes
your general health
Your doctor will talk with you about the treatments that are suitable for you. This includes the benefits and possible side effects of each treatment.
You may find it helpful to have a relative or friend with you when you see your doctor. And it may help to talk to your specialist nurse about any worries you have before you make your decision.
The most common treatments for ampullary cancer are surgery and chemotherapy.
You usually have surgery if the cancer hasn't spread, and you’re well enough for the operation. This is because surgery to remove ampullary cancer is a major operation.
You might have an operation called a pylorus preserving pancreaticoduodenectomy (pank-ree-at-ic-oh dew-oh-den-ek-tom-ee). Doctors also call this a PPPD. Or you might have an operation called a Whipple's procedure. Your doctor will talk with you about which operation they think is best for you.
Your surgeon normally removes part of your:
bile duct
pancreas
duodenum
They also remove your gallbladder and a number of near the cancer.
They then join your stomach, and the remaining part of your pancreas and bile duct to your small bowel.
Sometimes your surgeon also needs to remove the lower part of your stomach. This is where it joins your duodenum. Doctors also call this operation a pancreaticoduodenectomy.
The diagram below shows how your surgeon might join the remaining parts of your pancreas, stomach and bile duct during the operation.
Your pancreas is important for the digestion of food. It makes:
to control your blood sugar
pancreatic juice – this contains enzymes which help break down food and make it easier for your body to use
The remaining part of your pancreas should be able to make enough insulin after surgery. Your doctor will monitor your blood sugar in case this changes.
You may need to take an enzyme supplement after surgery. This is because the remaining part of your pancreas may not make enough pancreatic juice to help you digest your food properly.
You normally have a dietitian to support you with your eating and drinking after surgery. But it still might take time to return to a normal diet.
If you have a very early stage cancer, your surgeon may be able to do a smaller operation just to remove the ampulla. They then connect the bile duct and pancreatic duct straight to the duodenum. Doctors call this operation an ampullectomy.
You have a follow up appointment with your surgeon after your operation. This is to check you are recovering well and have no problems.
Find out more about having surgery for cancer
Chemotherapy uses anti cancer drugs to destroy cancer cells. These drugs are also called cytotoxic drugs. They circulate throughout the body in the bloodstream.
You might have chemotherapy:
after surgery to remove ampullary cancer
to control the cancer if it can't be removed, or has come back after surgery – this can help relieve symptoms
Your doctor might recommend chemotherapy after surgery to remove ampullary cancer. This is to help lower the chance of it coming back. It is called an adjuvant treatment.
You may have chemotherapy instead of surgery to remove the cancer if:
the cancer has spread elsewhere in your body
you’re not able to have an operation to remove the cancer
the cancer has come back after surgery to remove it
Unfortunately, if the cancer has spread elsewhere in your body, or has come back after treatment, the cancer is advanced. Cancer that has spread elsewhere is called secondary cancer. The aim of treatment is to control the cancer and reduce any symptoms. Your doctor will talk with you about your treatment options if this happens.
Read more about secondary cancer
Rarely, ampullary cancer cells may have gene changes (mutations). These may help the cancer grow and divide. Genes are made of . This is the genetic code that tells the body’s cells how to behave. Your doctor will check the cancer for these changes.
Your doctor might give you an immunotherapy drug called pembrolizumab if the cancer has DNA changes called:
Pembrolizumab is a checkpoint inhibitor. It blocks a protein called programmed cell death protein 1 (PD-1). PD-1 stops the immune system from attacking the cancer cells.
Find out more about having pembrolizumab
Even less often, ampullary cancer may have other gene changes. Targeted cancer drugs can control the cancer by targeting those changes. But unfortunately, not all gene changes have a drug that can target them.
Your doctor will talk to you about the best treatment if the cancer has one of these changes.
Find out more about your cancer drugs from our A to Z list
Once you have finished treatment you have regular follow up appointments with your doctor. They also ask you to have a CT scan and blood tests every 6 months.
Your doctor uses these appointments to check for side effects. And for signs of the cancer coming back. If there are no signs, you may start to see your doctor less often. If the cancer comes back, your doctor will talk with you about further treatment.
You normally have follow up for a number of years.
If you have any problems in between appointments, you can speak to your specialist nurse. They can arrange for you to see your doctor if necessary.
When you finish treatment, how often you have follow up appointments depends on:
how well the cancer is controlled
how you are feeling
Your doctor or specialist nurse can tell you how often your appointments will be.
They may also refer you to a symptom control or palliative care team. Someone from the team sees you or speaks to you regularly to help you cope with any symptoms you might have. How often you see them depends on what your symptoms are and how well they are controlled.
It can be difficult to research rare cancers like ampullary cancer. One reason is because there are very few people to take part in .
Researchers around the world are currently looking at:
treatment after surgery for people with ampullary cancers with different types of epithelial cells
how to better identify people who will benefit from chemotherapy after surgery
using endoscopy to remove early ampullary cancer
treatments for a very rare type of ampullary cancer called adenosquamous cell ampullary cancer
Unfortunately, these trials are not running in the UK. But there may be trials you can take part in that are looking at a wide range of cancers.
Speak to your doctor or specialist nurse if you are interested in taking part in a clinical trial.
Find out more about research and clinical trials
Coping with a diagnosis of rare cancer can be very difficult. Talking to your specialist nurse may help. They can talk with you about support services for you and your family. And they can refer you to specialist teams to help you cope.
Read more about coping with a rare cancer
For support and information, you can call the Cancer Research UK information nurses. They can give advice about who can help you and what kind of support is available. Freephone: 0808 800 4040 - Monday to Friday, 9am to 5pm.
Email the Cancer Research UK nurses
Talking to other people who have the same cancer can also help.
Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people.
Last reviewed: 28 Apr 2025
Next review due: 28 Apr 2028
Rare cancers affect a small number of people. In the UK and Europe experts say it is rare if fewer than 6 in 100,000 people are diagnosed each year. Find out more about rare cancer.
Find out about tests to diagnose cancer and monitor it during and after treatment, including what each test can show, how you have it and how to prepare.
Treatments can include surgery, radiotherapy and drug treatments (such as chemotherapy, hormone therapy or targeted cancer drugs). Find out about treatments and how to cope with side effects.
Coping with cancer can be difficult. There is help and support available. Find out about the emotional, physical and practical effects of cancer and how to manage them.
Some people have an increased risk of particular types of cancer because they have inherited a faulty gene.
This section has been written, reviewed and updated by Cancer Research UK’s Patient Information Web Team. Thank you to the expert medical professionals and people affected by cancer who have helped to review this information.
Professor John Bridgewater (Consultant Oncologist)
Professor Brian Davidson (Consultant Hepatopancreatobiliary Surgeon)
About Cancer generously supported by Dangoor Education since 2010. Learn more about Dangoor Education
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