Glucagonoma is a rare tumor involving the pancreas. Glucagon is a hormone produced by the pancreas that works with insulin to control the amount of sugar in your blood.

Glucagonoma tumor cells produce large amounts of glucagon, and these high levels create severe and life threatening symptoms.

If you have a tumor that produces large quantities of glucagon, it will affect many aspects of your health.

Glucagon balances the effects of insulin by regulating the amount of sugar in your blood. If you have too much glucagon, your cells don’t store sugar, and instead, sugar stays in your bloodstream.

Glucagonoma leads to diabetes-like symptoms and other severe symptoms, including:

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 percent of the time. Malignant glucagonomas spread into other tissues, usually the liver, and start interfering with the function of other organs.

It can be difficult to diagnose glucagonoma. Often, the symptoms appear to be caused by another condition, and it may be years before the correct diagnosis is made.

Diagnosis is initially made with several blood tests. High glucagon levels are the hallmark of this condition.

Other signs include:

  • high blood sugar
  • high levels of chromogranin A, which is a protein often found in carcinoid tumors
  • anemia, which is a condition in which you have a low level of red blood cells

Your doctor will follow up these tests with a CT scan of the abdomen to look for the presence of tumors.

About 75 percent of all glucagonomas are malignant. These tumors can spread throughout the body and invade other organs. Tumors are often large and can be 4 to 6 centimeters wide when they’re discovered. This cancer is often not discovered until it has spread to the liver.

Treating glucagonoma involves removing tumor cells and treating the effects of an excess of glucagon on your body.

It’s best to begin treatment by stabilizing the effects of excess glucagon. This often involves taking a somatostatin analog drug, such as an injection of octreotide (Sandostatin). Octreotide helps to counteract the effects of glucagon on your skin and improve skin rash.

If you’ve lost a great deal of weight, you may need an IV to help restore your body weight. High blood sugar can be treated with insulin and close monitoring of your blood glucose levels.

Your doctor may also give you an anticoagulant medication, or blood thinner. This prevents the formation of blood clots in your legs, also known as deep vein thrombosis.

If you’re at risk for deep vein thrombosis, a surgeon can place a filter in one of your large veins, the inferior vena cava, to prevent clots from reaching your lungs.

Once your general health has improved, the tumor will likely be surgically removed. This type of tumor rarely responds well to chemotherapy. Surgery is most successful if the tumor is caught while it’s still confined to the pancreas.

A surgeon may do exploratory surgery of your abdomen either laparoscopically (with small cuts to allow for cameras, lights, and tools) or by creating a larger open incision.

Most glucagonomas occur on the left side or tail of the pancreas. Removal of this section is called a distal pancreatectomy. The surgeon may also remove your spleen. When the tumor tissue is examined under a microscope, it’s difficult to tell whether it’s cancerous.

If it’s cancerous, your surgeon will remove as much of the tumor as possible to prevent it from spreading further. This may include part of the pancreas, local lymph nodes, and even part of the liver.

The complications of a glucagonoma include:

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.

Early detection can be difficult, so by the time glucagonoma is diagnosed, the cancer may already have spread to other organs, such as the liver. In this case, surgery isn’t effective.

Once a tumor is removed, the effect of excess glucagon decreases immediately. If the tumor is limited to just the pancreas, the 5-year survival rate is 55 percent, meaning that 55 percent of people live for at least 5 years after surgery.

There’s a 15 percent 5-year survival rate if the tumors can’t be removed surgically.

If treatment options are limited due to the extent of the tumor growth, it’s still important to take care of yourself. Reach out to a mental health professional or support group if you’re having trouble coping with your diagnosis.

Read this article in Spanish.