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A glucagonoma is a rare neuroendocrine tumor that originates almost exclusively in the pancreas and probably accounts for 1% of all neuroendocrine tumors. Malignant glucagonomas are islet cell pancreatic tumors that are discovered because of glucagonoma syndrome (in which the glucagonoma autonomously secretes glucagon), because of local mass effects, or incidentally. Glucagonomas originate from the alpha-2 cells of the pancreas. Unregulated production (overproduction) of peptide hormones and growth factors, which are not normally expressed in the tissue of origin, is characteristic of neuroendocrine tumors. Abnormal production of these bioactive peptides can lead to significant systemic toxic consequences and to the promotion of further tumor growth.

In 75-80% of cases, the glucagonoma starts in malignant form, and in 50% of these cases, metastasis exists at diagnosis. The tumor's presence is characterized by glucagon overproduction, weight loss, hyperglycemia, diabetes mellitus, hypoaminoacidemia, normochromic and normocytic anemia, and necrolytic migratory erythema (NME), which is the most characteristic clinical sign (as opposed to symptom) of this pathology


Glucose intolerance (body has problem breaking down sugars)

High blood sugar (hyperglycemia)


Excessive thirst (due to high blood sugar)

Frequent urination (due to high blood sugar)

Increased appetite.

Inflamed mouth and tongue.


There are no known direct causes of glucagonoma. If you have a family history of a syndrome called multiple endocrine neoplasia type 1 (MEN1), you have a greater risk for developing glucagonoma.

However, those who don’t have other risk factors can develop these tumors.

Glucagonomas are cancerous, or malignant, about 75 percentTrusted Source of the time. Malignant glucagonomas spread into other tissues, usually the liver, and start interfering with the function of other organs.

Risk factors

Extreme thirst.


Need to urinate frequently (including at night)

Increased appetite.

Inflammation of mouth or tongue.

Rash to skin on face, buttocks, lower limbs that frequently moves.

Crusty or scaly skin.

Raised sores.

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The complications of a glucagonoma include:

weight loss

chronic diarrhea

venous thrombosis

proximal muscle weakness

dilated cardiomyopathy



Deep vein thrombosis can cause blood clots to travel to the lungs, which can be fatal. If the tumor reaches the liver, it can eventually cause liver failure.

Therefore, it’s crucial that you contact your doctor if you suspect you have symptoms of glucagonoma, as early treatment is more effective.


If you have a tumor that has spread to other parts of your body, your glucagonoma syndrome symptoms will continue to be treated. Octreotide is a medication that can help regulate and prevent high levels of glucagon. Octreotide injections help reduce some glucagonoma side effects like high blood sugar and weight loss.