Treatment for glucagonomas

Glucagonoma is a type of neuroendocrine tumour (NET) that starts in the pancreas. The treatment you have depends on a number of things. This includes where the tumour is, its size and whether it has spread (the stage).

The first treatment you have is to control your symptoms. You then might have surgery to try to get rid of the tumour.

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.

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 tumour is and its size
  • whether it has spread
  • your general health
  • whether you have a rare inherited syndrome called multiple endocrine neoplasia 1 (MEN1)
Your doctor will discuss your treatment, its benefits and the possible side effects with you.

You are likely to have a clinical nurse specialist (or 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.

Surgery

Surgery is the only treatment that can cure a glucagonoma. The type of surgery you have depends on the size of the tumour, where it is and whether it has spread to other parts of the body such as the liver.

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 in your tummy (abdomen) to remove the glucagonoma. They might also remove the nearby lymph nodes. 

You might have surgery to remove:

  • just the tumour (enucleation)
  • the narrowest part of the pancreas and the body of the pancreas (distal pancreatectomy)
  • the whole of the pancreas (total pancreatectomy)
  • the widest part of the pancreas, the duodenum, gallbladder and part of the bile duct (pylorus preserving pancreaticoduodenectomy or PPPD for short)
  • the widest part of the pancreas, duodenum, gallbladder, part of the bile duct and part of the stomach (Whipple’s operation)
Diagram showing the position of the liver, gallbladder and pancreas

If the glucagonoma has spread to the liver, you might be able to have the liver tumour removed at the same time you have the main surgery. Your surgeon may remove just the tumour, or part of the liver too.

Remember to click back to return to the NET section. The rest of the information in the pancreatic section will not apply to you. 

Treatments to help with symptoms

You have treatments to help with symptoms before you have surgery. You might also have these treatments if you can’t have surgery to remove the tumour, or if it comes back after surgery.

These treatments can control the symptoms and help you feel better.

Symptoms are usually caused by the increase in the amount of glucagon in the body. So you might have drugs to reduce the amount of glucagon you make. 

You might also have:

Amino acids 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 with a common symptom of glucagonoma which is a skin rash called necrolytic migratory erythema (NME).

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 insulin injections.

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.

You may have somatostatin analogues to try to slow down the tumour and help with symptoms. They include: 

  • octreotide (Sandostatin)
  • lanreotide (Somatuline)

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%).

If the glucagonoma has spread to the liver, you might be able to have the liver tumour removed at the same time. Your surgeon may remove just the tumour, or part of the liver too. 

Radiofrequency ablation (RFA) uses heat made by radio waves to kill tumour cells. You might have this if the NET has spread to the liver.

You might have this treatment if the NET has spread to the liver. 

Trans arterial embolisation (TAE) means having a substance such as a gel or tiny beads to block the blood supply to the liver NET. It is also called hepatic artery embolisation.

You may also have a chemotherapy drug to the liver at the same time. This is called trans arterial chemoembolisation (TACE). But doctors don't know for sure whether adding chemotherapy is better than having embolisation alone for NETs that have spread to the liver. 

Embolisation and chemoembolisation work in two ways:

  • it reduces the blood supply to the tumour and so starves it of oxygen and the nutrients it needs to grow
  • it gives high doses of chemotherapy to the tumour without affecting the rest of the body

You may have a type of internal radiotherapy called peptide receptor radionuclide therapy (PRRT). Internal radiotherapy means having radiotherapy from inside the body (as a drip into your bloodstream). 

PRRT uses a radioactive substance called lutetium-177 or yttrium-90 attached to a somatostatin analogue. 

You may have PRRT if:

  • your NET has spread to other parts of the body
  • you can’t have surgery
  • your NET has receptors on the outside of them called somatostatin receptors (you have special scans called octreotide or gallium PET scans to check for this)

Cancer cells have changes in their genes (DNA) that make them different from normal cells. These changes mean that they behave differently. Targeted drugs work by ‘targeting’ the differences that a cancer cell has and destroying them. 

You may have 2 types of targeted drugs called everolimus and sunitinib.

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy tumour cells. You may have chemotherapy if the NET has spread to the liver or to other parts of your body. 

The most common chemotherapy drugs used are:

  • streptozotocin or temozolomide
  • fluorouracil or capecitabine
  • doxorubicin

Interferon is also called interferon alfa. You may have it if the glucagonoma has spread to other parts of the body. And other treatments have stopped working. 

You may have interferon alone or with somatostatin analogues. 

Clinical trials

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.

Last reviewed: 
05 Aug 2021
Next review due: 
05 Aug 2024
  • Functioning and nonfunctioning pNENs
    K Daskalakis 
    Current Opinion in Endocrine and Metabolic Research, 2021. Vol 18, June 2021, Pages 284-290

  • Glucagonoma and the glucagonoma syndrome – cumulative experience with an elusive endocrine tumour
    R Eldor and others
    Clinical Endocrinology, 2011. Vol 74, Pages 593-598

  • Rapid clearance of necrolytic migratory erythema following intravenous administration of amino acids
    E Thomaidou and others
    JAMA Dermatology, 2016 Vol 152, Issue 3, Pages 345-346

  • Glucagonoma and the glucagonoma syndrome
    Emily Bergsland
    UpToDate, 2021

  • Neuroendocrine Tumours: Diagnosis and Management
    S Yalcin and K Oberg
    Springer, 2015

  • ENETS consensus guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes
    R Jensen and others
    Neuroendocrinology, 2012. Vol 95, Pages 98-119

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