What are the other Names for this Condition? (Also known as/Synonyms)

• Syndrome due to Glucagon Secreting Tumor

What is Glucagonoma Syndrome? (Definition/Background Information)

• Glucagonoma Syndrome is a rare condition causing skin lesions that occur due to increased glucagon in blood. It is usually caused by glucagonoma (tumor of the pancreas)
• Glucagonoma is caused by an increase in glucagon hormone produced by the alpha cells of the pancreas. Hence, Glucagonoma Syndrome usually occurs due to tumors of the pancreatic alpha cells
• The syndrome occurs worldwide and is commonly observed in older individuals. One of the risk factors for the condition is having a family history of multiple neuroendocrine neoplasia type 1 (MEN 1). Nevertheless, the exact cause of Glucagonoma Syndrome is unknown
• The skin can be affected in a specific pattern called superficial migratory necrotic erythema (MNE). The skin nodules can show increased pigmentation, scaling, blister formation, and/or ulceration
• The development of diabetes, blindness, and deep vein thrombosis can occur as complications due to Glucagonoma Syndrome. The tumor is usually slow-growing and often may have already spread to the liver at the time of diagnosis (metastasis to liver)
• Usually, chemotherapy is not very effective against Glucagonoma Syndrome. The reason for this is not known. Hence, surgery is usually the preferred treatment option
• The prognosis is dependent upon the stage of the pancreatic tumor. If the tumor is only present in the pancreas, then the 5-year survival rate is 85%

Who gets Glucagonoma Syndrome? (Age and Sex Distribution)

Information on prevalence, male-female ratio, age of affliction, racial predilection, and geographical restriction/distribution information:

• Glucagonoma Syndrome can occur at any age, but is frequently seen in individuals over the age of 50 years
• Both males and females can be affected by the condition
• It is seen to occur worldwide and no racial or ethnic preference is observed

What are the Risk Factors for Glucagonoma Syndrome? (Predisposing Factors)

A positive family history of multiple neuroendocrine neoplasia type 1 (MEN 1, a disorder affecting the endocrine system) is an identified risk factor for Glucagonoma Syndrome.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Glucagonoma Syndrome? (Etiology)

• The exact cause of development of Glucagonoma Syndrome is unknown. They are thought to occur due to abnormal mutations in certain genes
• Research is being currently performed to identify the relevant cause factors

What are the Signs and Symptoms of Glucagonoma Syndrome?

The signs and symptoms of Glucagonoma Syndrome include:

• The skin can be affected in a specific pattern called superficial migratory necrotic erythema (MNE), a characteristic sign of Glucagonoma Syndrome. It is important to note that MNE can occur in individuals, even without the presence of a glucagon-secreting tumor of the pancreas
• Skin nodules can show increased pigmentation. The affected skin can show scaling, blister formation, and/or ulceration
• The skin of the fingertips can show ulcers
• Skin lesions can occur around the mouth (perioral) and around the genital area (perigenital)
• The tumor is usually slow growing
• The condition can cause weight loss and result in malnutrition

Other signs and symptoms include:

• Increased thirst and hunger
• Frequent urination
• Diarrhea
• Increased tendency to form blood clots in legs
• Depression

How is Glucagonoma Syndrome Diagnosed?

Glucagonoma Syndrome is diagnosed using the following methods:

• A thorough physical examination, correlated with a complete medical history
• The following lab tests may be recommended:
  • Complete blood count (CBC)
  • Glucagon levels by radioimmunoassay (RIA)
  • Fasting blood glucose level
  • Serum fasting insulin level
  • Serum fasting glucagon level
  • Serum level of chromogranin A
  • Albumin blood test
  • Total serum protein with albumin/globulin ratio
  • Zinc blood levels test
• A CT scan or ultrasound of the abdomen may reveal the tumor of the pancreas

Note:

• A complete blood count may reveal anemia (low hemoglobin)
• Blood test may show decreased protein levels, decreased serum albumin and globulin proteins
• Lab results may show increased fasting plasma glucagon and increased blood glucose. They can also show decreased serum zinc levels

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Glucagonoma Syndrome?

Complications due to Glucagonoma Syndrome could include:

• The development of diabetes, blindness, and deep vein thrombosis
• Metastasis to liver: It is often observed to have spread to the liver at the time of diagnosis
• The skin lesions can cause cosmetic issues

How is Glucagonoma Syndrome Treated?

The treatment measures for Glucagonoma Syndrome may include:

• Surgical procedures are preferred over chemotherapy, since chemotherapy is found to be ineffective against Glucagonoma Syndrome
• Total parenteral nutrition for malnutrition (TPN)
• Some studies have shown zinc replacement therapy to be beneficial
• Treatment with octreotide can help in decreasing the adverse effect of glucagon secretion by inhibiting its release from the pancreas
• Superficial migratory necrotic erythema, which is caused by the Glucagonoma tumor, is often difficult to treat

How can Glucagonoma Syndrome be Prevented?

• Currently there are no definitive methods available to prevent Glucagonoma Syndrome
• Individuals who have a family history of multiple neuroendocrine neoplasia type 1 may benefit from genetic counseling
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

What is the Prognosis of Glucagonoma Syndrome? (Outcomes/Resolutions)

• The prognosis is dependent upon the stage of the pancreatic tumor. If the tumor is only present in the pancreas, then the 5-year survival rate is 85%
• In many cases, migratory necrotic erythema disappears after surgical removal of the pancreatic tumor
• However, in a majority of cases, this tumor has already spread to the liver at the time of diagnosis

Additional and Relevant Useful Information for Glucagonoma Syndrome:

• Less than 500 cases of Glucagonoma Syndrome have been reported around the world
• It is important to note that multiple neuroendocrine neoplasia type 1 (MEN 1) is different from superficial migratory necrotic erythema (MNE)