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Glucagonoma Syndrome - Symptoms & Treatment


The glucagonoma syndrome is an uncommon clinicopathologic entity. It is very rare and affects adults over the age of 50. The cause is unknown, but genetic factors play a role in some cases. Glucagonoma is usually malignant, which means that it has a tendency to spread and get worse. Glucagonoma syndrome is due to a slow-growing cancerous tumour located in the alpha cells of the pancreas. The syndrome also illustrates the dramatic effects that can result from derangements in the regulation of basic metabolic pathways. Another feature of the syndrome that is noteworthy is the high rate of thromboembolic complications and consequent pulmonary embolisms; this is dangerous for many patients who can succumb to it. It may affect any site but it most often affects the genital and anal region, the buttocks, groin and lower legs. The rash fluctuates in severity. Initially there is a ring-shaped red area that blisters, erodes and crusts over. It is characterized by weight loss, necrolytic migratory erythema, diabetes, stomatitis, and diarrhea. The skin can be a mirror of various internal diseases. Some of the skin lesions if recognised early, may enable both the dermatologists and the physicians alike in the early detection and treatment of the more serious internal diseases.

Causes of Glucagonoma syndrome

The common causes and risk factor's of Glucagonoma syndrome include the following:

  • A slow-growing cancerous tumour located in the alpha cells of the pancreas.
  • Genetic factors.
  • A family history of multiple endocrine neoplasia type I.

Symptoms of Glucagonoma syndrome

Some sign and symptoms related to Glucagonoma syndrome are as follows:

  • Weight loss.
  • Nocturnal (nighttime) urination.
  • Diarrhea.
  • Increased appetite.
  • Excess thirst.
  • Characteristic brown-red skin rash.

Treatment of Glucagonoma syndrome

Here is list of the methods for treating Glucagonoma syndrome:

  • Treatment includes surgical resection, leading to prompt resolution of the rash, although intravenous amino-acids and somatostatin/octreotide may be necessary for symptom palliation.
  • Chemotherapy treatments including streptozocin, fluorouracil, dacarbazine and octreotide may also help some patients.
  • Intermittent infusion of amino acids and fatty acids via peripheral intravenous access can provide long-term improvement of Glucagonoma syndrome.

 

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