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Pancreatic neuroendocrine cancer

Glucagonoma

Glucagonomas are rare neuroendocrine tumours (NETs). They start in glucagon making cells of the pancreas. They usually make large amounts of glucagon which can raise your blood sugar levels.

What are neuroendocrine tumours (NETs)?

Doctors sometimes group NETs depending on whether they make and release abnormal levels of hormones:

  • Non functioning NETs make and release normal levels of hormones.

  • Functioning NETs make and release abnormal levels of hormones. 

Glucagonomas are a type of functioning NET. They usually make large amounts of glucagon. Glucagon is a hormone that raises the blood sugar levels in your body.

They are also called:

  • gastro entero pancreatic neuroendocrine tumours (GEP NETs)

  • pancreatic neuroendocrine tumours (pNETs)

  • islet cell tumours - because the cells they start in are also called islet cells

Find out more about NETs

About the pancreas

The pancreas is part of our ​​.

Diagram showing where the pancreas is in the body in relation to the other organs. This includes the stomach, liver, bowel and gallbladder.

The pancreas is quite high up in the tummy (abdomen). It lies across your body where the ribs meet at the bottom of the breastbone, just behind your stomach. It is about 6 inches long (15 centimetres) and shaped like a leaf. 

The pancreas has 3 parts:

  • the wide part is the head

  • the thin end is the tail

  • the part in the middle is the body

Diagram showing 3 parts of the pancreas.

The pancreatic duct is a tube that collects the digestive juices made by the pancreas. It carries them into the first part of the small bowel (duodenum).

How common is glucagonoma?

Glucagonomas are very rare. Less than 1 person in every 10 million develop a glucagonoma every year.

Where does glucagonoma start?

Glucagonomas start in the pancreas. Most start in the tail of the pancreas.

Some glucagonomas grow slowly and don't spread to other parts of the body. But others do spread (metastasise). Over 50 out of 100 glucagonomas (over 50%) have spread to other parts of the body at diagnosis.

The most common places where glucagonomas spread to are the:

  • liver

  • bones

Symptoms of glucagonoma

Glucagonomas usually make the hormone glucagon. You might have symptoms caused by the increase in the amount of glucagon in your body. Glucagon helps to raise the level of blood sugar in your body.

Symptoms of glucagonomas usually develop slowly. Some people are only diagnosed with a glucagonoma some years after developing their first symptom.

Symptoms might include a skin rash. This usually starts with small circles of redness which develop into itchy, painful blisters. The rash is called necrolytic migratory erythema (NME). It can affect most parts of the body but is more common in the:

  • buttocks

  • groin

  • back passage (anal area)

  • sexual organs such as the penis and vagina (genitals)

  • lower part of the legs

Other symptoms include:

  • unexplained weight loss

  • high blood sugars which can cause symptoms such as thirst, passing a lot of urine and weakness

  • mouth ulcers

  • diarrhoea

  • blood clots

  • mood changes including feeling depressed and agitated

  • low levels of red blood cells (anaemia)

Seeing your GP and referral to a specialist

If you have symptoms, you usually see your GP first. They might organise some tests. Your GP will decide if you need to see a specialist. For example, they might refer you to a skin specialist (dermatologist) if you have a skin rash.

Glucagonomas are rare tumours. So your doctor might ask you to have tests that check for other conditions first.

If your doctor suspects cancer, you have tests to check the type of cancer you have. If tests show that you have a glucagonoma, your specialist will refer you to a team of doctors and specialist nurses who have expertise in treating NETs.

Find out more about seeing a specialist

Tests to diagnose glucagonoma

Your GP and specialist arrange tests to diagnose your cancer. Tests also show the size of the cancer and whether it has spread. Some of the tests help your doctor plan your treatment.

Tests might include:

  • checking your blood glucagon level

  • blood tests to check your general health and to check the levels of other substances raised by NETs

  • ​ of a skin rash

  • radioactive scans – these are ​​ or Tektrotyd scans, and gallium PET scans

  • an ​​ scan of your tummy to look at your stomach, pancreas and ​​ , and to take biopsies

Read more about the tests to diagnose NETs

Grades and stages of glucagonoma

Grade

The ​​ looks at a sample of glucagonoma cells under a microscope. They look at:

  • how abnormal the cancer cells look – doctors call this differentiation

  • how quickly or slowly they are dividing and growing – this is grading

Glucagonomas are well differentiated cancers. This means the NET cells look abnormal. But they still have some similarities to normal neuroendocrine cells.

Stage

You have tests to find out the size of the glucagonoma and whether it has spread. This is your stage. Doctors use your stage to plan your treatment.

There are different ways to stage pancreatic NETs. Doctors usually use the TNM system or number stages. There are 4 main stages of pancreatic NETs - stage 1 to stage 4.

Read more about the grades and stages of pancreatic NETs

Risks and causes of glucaganoma

A risk factor is anything that increases your risk of getting a disease. 

We don’t know what causes most neuroendocrine cancers. But there are some risks factors that can increase your risk of developing it. Having one of these risk factors doesn’t mean that you will definitely develop a cancer. 

The risk factors for pancreatic NETs include:

  • inherited conditions such as ​ and ​

  • having a family history of cancer 

Read more about the risks and causes of neuroendocrine cancer

Treatment for glucagonoma

The first treatment you have is to control your symptoms. You then might have surgery to try to remove the NET.

But surgery isn’t always possible. Some glucagonomas may have already started to spread when you are diagnosed. Or you may not be well enough to have it. You continue to have treatment to help your symptoms if surgery isn’t an option.

You might have other treatments if surgery isn’t an option.

Deciding which treatment you need

A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT).

The treatment you have depends on:

  • where the NET is and its size

  • how many tumours you have

  • whether it has spread to other parts of the body

  • your general health and fitness

  • the symptoms you have

Your doctor will discuss your treatment. And tell you about its benefits and the possible side effects.

You are likely to have a clinical nurse specialist (CNS). They go to the MDT meetings. They can help answer your questions and support you. They are often your main point of contact throughout your treatment.

Your MDT also consider whether you have MEN 1. This might affect treatment decisions.

Click on the headings below to read more about the different treatment options.

Follow up after treatment

You usually have follow up appointments after treatment. This is to check how you are and see whether you have any problems or worries. 

You might also have tests on some visits. The tests might include:

  • blood tests

  • an MRI or CT scan

How often you see your doctor depends on:

  • the grade of your NET

  • whether it has spread to other parts of the body

  • what treatment you have had

  • any side effects you might have

It is important you contact your doctors if you have any worrying symptoms between appointments.

Read more about follow up

Survival (prognosis) for glucagonoma

The best person to talk to you about your outlook (​) is your doctor. Not everyone wants to know. People cope differently with cancer and want different information.

Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.

Your prognosis will depend on the type of NET you have and what ​​ it is.

Read more about survival for neuroendocrine cancers

Coping with neuroendocrine cancer

You might have physical changes to your body because of your cancer or the treatment. These changes can be hard to cope with and can affect the way you feel about yourself. It can help to talk to friends and family. Or join a support group to meet people in a similar situation.

We have a discussion forum called Cancer Chat. It is a place for anyone affected by cancer. You can share experiences, stories and information with other people with cancer.

Visit Cancer Chat

You might need practical advice about benefits or financial help. There is help and support available. There are also organisations to support and provide information to people affected by neuroendocrine cancer.

We have information about living and coping with a neuroendocrine cancer

Last reviewed: 11 Mar 2025

Next review due: 11 Mar 2028

Pancreatic neuroendocrine cancer main page

There are different types of neuroendocrine cancer that develop in the pancreas. Pancreatic neuroendocrine tumours (NETs) include insulinoma, gastrinoma, somatostatinoma, glucagonoma and VIPoma.

Grades, types and stages of pancreatic neuroendocrine cancer

Grade means how quickly or slowly the cancer cells are dividing and growing. The stage of a neuroendocrine cancer tells you its size and whether it has spread. There are many different types.

Treatment for neuroendocrine cancer

Treatment depends on the type of neuroendocrine cancer you have, where it is, its size and whether it has spread (the stage).

Survival for neuroendocrine cancer

Survival (prognosis) depends on several factors. This includes your type of neuroendocrine cancer, where it is in your body, and whether it has spread.

Living with neuroendocrine cancer

Practical and emotional support is available to help you cope with neuroendocrine cancer.

Neuroendocrine cancer main page

Neuroendocrine cancers develop in cells of the neuroendocrine system. They can develop in different parts of the body including the lungs, stomach, pancreas and bowel.

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