What is the definition of Glucagonoma?

Glucagonoma is a very rare tumor of the islet cells of the pancreas, which leads to an excess of the hormone glucagon in the blood.

What are the alternative names for Glucagonoma?

MEN I - glucagonoma

What are the causes for Glucagonoma?

Glucagonoma is usually cancerous (malignant). The cancer tends to spread and get worse.

This cancer affects the islet cells of the pancreas. As a result, the islet cells produce too much of the hormone glucagon.

The cause is unknown. Genetic factors play a role in some cases. A family history of the syndrome multiple endocrine neoplasia type I (MEN I) is a risk factor.

What are the symptoms for Glucagonoma?

Symptoms of glucagonoma may include any of the following:

  • Glucose intolerance (body has problem breaking down sugars)
  • High blood sugar (hyperglycemia)
  • Diarrhea
  • Excessive thirst (due to high blood sugar)
  • Frequent urination (due to high blood sugar)
  • Increased appetite
  • Inflamed mouth and tongue
  • Nighttime (nocturnal) urination
  • Skin rash on face, abdomen, buttocks, or feet that comes and goes, and moves around
  • Weight loss

In most cases, the cancer has already spread to the liver when it is diagnosed.

What are the current treatments for Glucagonoma?

Surgery to remove the tumor is usually recommended. Chemotherapy may be done when surgery is not possible.

What are the support groups for Glucagonoma?

You can ease the stress of illness by joining a cancer support group. Sharing with others who have common experiences and problems can help you not feel alone.

What is the outlook (prognosis) for Glucagonoma?

Approximately 60% of these tumors are cancerous. It is common for this cancer to spread to the liver. Only about 20% of people can be cured with surgery.

If the tumor is only in the pancreas and surgery to remove it is successful, people have a 5-year survival rate of 85%.

What are the possible complications for Glucagonoma?

The cancer can spread to the liver. High blood sugar level can cause problems with metabolism and tissue damage.

When should I contact a medical professional for Glucagonoma?

Contact your provider if you notice symptoms of glucagonoma.

Endocrine

REFERENCES

Asban A, Patel AJ, Reddy S, Wang T, Balentine CJ, Chen H. Cancer of the endocrine system. In: Niederhuber JE, Armitage JO, Kastan MB, Doroshow JH, Tepper JE, eds. Abeloff's Clinical Oncology. 6th ed. Philadelphia, PA: Elsevier; 2020:chap 68.

National Cancer Institute website. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq. Updated January 2, 2020. Accessed March 17, 2021.

Newey PJ, Thakker RV. Multiple endocrine neoplasia. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 42.

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