Ampullary cancer
The ampulla is part of the 
It is also called the ampulla of Vater. It is where bile and pancreatic juices drain into the first part of the small bowel. Doctors call this part of the bowel the duodenum.
Ampullary cancer is when abnormal cells in the ampulla divide in an uncontrolled way. It is also called ampulla of Vater cancer.
The ampulla
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.
Where does ampullary cancer start?
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.
How common is ampullary cancer?
Ampullary cancer is very rare. In the UK, around 550 people are diagnosed with ampullary cancer each year.
These statistics are based on the average annual number of new cases of ampullary cancer (ICD10 C24.1) in the UK between 2017 and 2019.
Risks and causes of ampullary cancer
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.
Symptoms of ampullary cancer
There are many other conditions that cause these symptoms. Most of them are much more common than ampullary cancer.
Yellowing of the skin and the white of your eyes (jaundice)
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
See your GP urgently or call NHS 111 if you have jaundice.
Blood in your poo
This might be bright red, or it might be almost black if the blood has been in your stomach for a while. You should see a doctor urgently or call 111 if you have blood in your poo or have bloody loose stools (diarrhoea).
You should go to A&E or call 999 if the bleeding isn’t stopping or there is lots of blood.
Blood in your sick
This might be bright red or it may look dark brown, like used coffee grounds.
You should go to A&E or call 999 if you have blood in your sick and feel unwell or have black poo. If you have stopped bringing up blood and have no other symptoms you should call 111 or see your GP urgently
Inflammation of the pancreas
This is called pancreatitis. It can cause you to have a fever, feel or be sick and have pain in your upper tummy (abdomen). This pain may go around to your back
See your GP or call NHS 111 urgently if you have these symptoms.
Other symptoms
- pain in your abdomen not caused by pancreatitis
- loose poo (diarrhoea)
- unexplained weight loss
See your GP if you have any of these symptoms. Although your symptoms are unlikely to be ampullary cancer, it is important to get them checked by a doctor.
Getting diagnosed with ampullary cancer
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
ultrasound scan of your abdomen - a
CT scan of your chest, abdomen and the area between your hips (pelvis) - an
endoscopy 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.
Treatment 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
Treatment options
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.
Surgery
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.
PPPD
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.
Whipple's procedure
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.
Having part of your pancreas removed
Your pancreas is important for the digestion of food. It makes:
insulin 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.
Surgery to remove the ampulla
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.
Follow up after surgery
You have a follow up appointment with your surgeon after your operation. This is to check you are recovering well and have no problems.
Chemotherapy
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
Chemotherapy after surgery
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.
Chemotherapy to control the cancer
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.
Immunotherapy and targeted cancer drugs
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.
Immunotherapy
Your doctor might give you an immunotherapy drug called pembrolizumab if the cancer has DNA changes called:
microsatellite instability (MSI-high) mismatch repair deficiency (dMMR)
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.
Targeted cancer drugs
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.
Follow up for ampullary cancer
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.
After treatment for advanced cancer
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.
Research into ampullary cancer
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.
Coping with ampullary cancer
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.
Cancer Research UK nurses
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.
Cancer Chat
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.
Rare Cancer Alliance
The Rare Cancer Alliance offer support and information to people with rare cancers.
