How Do You Treat Glucagonoma?

Glucagonoma Care: Early Detection, Therapy and Prognosis

Glucagonoma is a rare type of pancreatic neuroendocrine tumour that causes the overproduction of glucagon, a hormone that raises blood sugar levels. This excess production can lead to significant metabolic disturbances and distinct skin-related symptoms.

Early diagnosis is crucial, as it significantly improves treatment outcomes and helps manage complications more effectively. The treatment approach depends on the stage of the disease. For localised tumours, surgical removal is the primary treatment. For advanced or inoperable cases, or to manage symptoms before surgery, a multidisciplinary approach is used, combining medical therapy and nutritional support. 

What are the treatment options for glucagonoma?

Treatment for glucagonoma generally involves a combination of medication and surgery, depending on the stage and spread of the tumour. Let us explore the treatment options in detail below:

• Medical Therapy : Patients with glucagonoma have an increased risk of developing blood clots, such as deep vein thrombosis (DVT). Doctors will assess this risk and may prescribe anticoagulants (blood thinners) if the benefits are deemed to outweigh the risks of bleeding. 
  Targeted therapies such as somatostatin analogues (e.g., octreotide or lanreotide) may help control hormone secretion and reduce tumour growth in cases where surgery is not feasible. In some advanced cases, chemotherapy or peptide receptor radionuclide therapy (PRRT) may also be considered.
• Surgery : Surgical removal of the tumour remains the most effective treatment when the tumour is localised and resectable. A distal pancreatectomy is commonly performed if the tumour is located in the tail of the pancreas. Complete surgical removal can significantly improve long-term outcomes and, in some cases, may be curative. However, surgery is not always an option if the tumour has metastasised or involves critical blood vessels.
• Prognosis and Follow-Up : The prognosis of glucagonoma largely depends on whether the tumour is localised or has metastasised. Surgical resection improves the survival rate significantly when detected early. Continuous follow-up care, including regular imaging and blood tests, is essential to monitor for recurrence or progression.

How Common is Glucagonoma?

Glucagonoma is extremely rare. According to the National Institutes of Health (NIH), the incidence is approximately 1 in 20 million people per year worldwide, although earlier data suggested 1 in 1 million. Most cases are diagnosed in individuals between 50 and 70 years of age, and women are slightly more frequently affected than men.

What are the signs and symptoms of glucagonoma?

Glucagon plays a key role in balancing the effects of insulin by increasing blood glucose levels. Excess glucagon leads to a variety of symptoms, most notably:

• Necrolytic migratory erythema (NME): A distinctive skin rash found in over 70–90% of patients, often on the belly, face, buttocks, or feet.
• High blood sugar: Symptoms may mimic diabetes, such as excessive hunger, thirst, and frequent urination.
• Unexplained weight loss
• Persistent diarrhoea
• Blood clots in the legs (DVT)

In addition to these, glucagonoma can cause neurological and psychiatric symptoms, including:

• Dementia
• Depression
• Agitation
• Ataxia
• Hyperreflexia
• Psychosis
• Paranoid delusions

What are the criteria for glucagonoma?

The exact cause of glucagonoma is unknown. However, approximately 10% of cases are associated with an inherited condition called multiple endocrine neoplasia type 1 (MEN1).

Diagnosis usually involves a combination of:

• Blood tests: Including fasting glucose and glucagon levels
• Imaging tests: Such as MRI, CT scan, or somatostatin receptor scintigraphy
• Biopsy: To confirm the diagnosis through histological examination

Early detection is essential to prevent complications and metastasis.

Timely treatment of glucagonoma is essential for improving patient outcomes. Surgical resection offers the best prognosis when feasible. For advanced or inoperable cases, medical therapy and targeted treatments help manage symptoms and slow tumour progression. Continuous follow-up, early intervention, and a team-based approach ensure the best chance of long-term survival and quality of life.