Glucagonoma
Glucagonomas are rare neuroendocrine tumours (NETs). They start in the glucagon making cells of the pancreas. These cells are also called islet cells.
Neuroendocrine tumours (NETs) are a group of neuroendocrine cancers. Doctors also use the term neuroendocrine neoplasm (NEN) for neuroendocrine cancer.
NETs start in 
and can develop in different parts of the body.
NETs can be slow or fast growing. The cancer cells look abnormal. But they still have some similarities to normal neuroendocrine cells.
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 usually make large amounts of glucagon. Glucagon is a hormone that raises the blood sugar levels in your body.
Glucagonomas are a type of functioning NET. They are also called gastro entero pancreatic neuroendocrine tumours (GEP NETs). Or pancreatic neuroendocrine tumours (pNETs).
The pancreas
The pancreas is part of our .
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
The pancreatic duct is a tube that collects the digestive juices made by the pancreas. It carries them into the first part of the 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
lymph nodes - bones
- lungs
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)
These symptoms could be due to a glucagonoma, but can also be caused by other more common medical conditions. It’s important to get them checked by a doctor.
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.
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
- a blood test to check for a rare inherited condition called
multiple endocrine neoplasia 1 (MEN1) a CT scan an MRI scan - radioactive scans – these are octreotide scans (or
octreoscans ) and gallium PET scans - an
endoscopic ultrasound scan of your tummy to look at your stomach, pancreas and bile ducts , and to take biopsies
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.
There are 3 grades of glucagonomas – grade 1, 2 and 3. Grade 1 grow slowly, and grade 3 grow quickly. Grade 2 grow at a moderate speed.
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.
Risks and causes
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:
- an inherited condition called multiple endocrine neoplasia 1 (MEN1)
- having a family history of 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.
Types of treatment for glucagonoma
Symptoms are usually caused by the increase in glucagon levels in your body. So you might have drugs to reduce the amount of glucagon you make.
You might have:
- an
amino acid drip - drugs to control blood sugar levels
- drugs called
somatostatin analogues
Amino acid drip
People with glucagonomas often have low levels of amino acids. Amino acids are the building blocks of proteins. You usually have amino acids as a drip into a vein. You might have 1 or more drips. This might help if you have a skin rash.
Drugs to control blood sugar levels
You have drugs to control the blood sugar level if it becomes too high. This is usually tablets, but you might also have injections.
Blood clots
Your doctor might also suggest you have medicine to lower your risk of . These are called anticoagulants.
Somatostatin analogues
Somatostatin is a protein made naturally in the body. It does several things including slowing down the production of hormones. Somatostatin analogues are man made versions of somatostatin.
The most common drugs are:
- octreotide
- lanreotide
Support with weight loss and diet
You might see a dietician if you have lost a lot of weight. They will give you advice about what you are eating and drinking. You might need to have:
- vitamin or mineral supplements
- drip or tube feeding if you have lost a lot of weight
The type of surgery you have depends on the size of the NET and where it is.
Some of these are major operations and there are risks. But if the aim is to try to cure your glucagonoma, you might feel it is worth some risks. Talk to your doctor about the risks and benefits of your surgery.
You usually have open surgery. This means your surgeon makes a large cut (incision) in your tummy (abdomen) to remove the glucagonoma. They might also remove the nearby lymph nodes.
There are different operations:
Enucleation means the surgeon removes just the tumour. They leave the healthy pancreatic tissue behind.
A distal pancreatectomy means the surgeon removes:
- the narrowest part of the pancreas
- the body of the pancreas
A pylorus preserving pancreaticoduodenectomy (PPPD) means the surgeon removes:
- the widest part of the pancreas
- the duodenum
- the gallbladder
- part of the bile duct
A Whipple’s operation means the surgeon removes:
- the widest part of the pancreas
- duodenum
- gallbladder
- part of the bile duct
- part of the stomach
If the cancer has spread to the liver, your surgeon might remove the liver tumours at the same time as the main surgery. Your surgeon may remove just the tumour or part of the liver.
Surgery to remove part of the tumour
Removing part of the tumour can reduce your symptoms. Your doctor will only suggest surgery if they think it’s possible to remove most of the tumour (at least 90%). They also call this debulking surgery.
You might have other treatments if:
- you can’t have surgery to remove the NET
- the NET has spread to another part of the body
- the NET came back after the initial treatment
These treatments can control your symptoms and help you feel better but won’t get rid of the NET. There are a number of different treatment options:
somatostatin analogues - drugs include octreotide and lanreotide- peptide receptor radionuclide therapy (PRRT) - this uses a radioactive medicine to treat the NET
chemotherapy – drugs include temozolomide, fluorouracil, capecitabine, streptozotocintargeted cancer drugs – drugs include everolimus and sunitinib
For cancer that has spread to your liver you might have:
- trans arterial embolisation (TAE) to cancer in your liver
radiofrequency ablation - surgery to remove cancer from your liver
- a liver transplant – this is a rare treatment for NETs
Doctors are always trying to improve treatments and reduce the side effects. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or to look at different combinations of existing treatments.
Follow up
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.
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.
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.
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.
