Glucagonoma
Glucagonoma Causes
The exact cause of glucagonoma is unknown, but may be linked to genetics. Those whose family history includes multiple endocrine neoplasia type 1 are at risk.
Glucagonoma Definition
Glucagonoma is a tumor affecting the pancreas' alpha cells, causing extreme surplus production of the hormones insulin and glucagons. The malignant and fast-spreading nature of the disease affects these alpha cells, causing the overproduction of hormones.
Glucagonoma Diagnosis
Blood tests are significant in diagnosing glucagonoma. If they show abnormally low concentrations of essential fatty acids, zinc, and amino acids, this means the patient has glucagonoma. A blood serum glucagon count of at least 1000 pg/ml is also equivalent to a glucagonoma diagnosis. Certain procedures may aid in localizing the tumor, including CT, angiography, PET, MRI, as well as an endoscopic ultrasound.
Glucagonoma Symptoms and Signs
Symptoms of glucagonoma include an inflamed tongue and mouth, increased urination and nocturnal urination, increased appetite, excessive thirst, unintentional weight loss, skin rash that may occur on the abdomen, face, buttocks, or lower extremities. The insulin and glucagon imbalance in the body may lead to diabetes mellitus. This occurs in 90% of patients who have glucagonoma. Necrolytic migratory erythema or NME is considered a classical symptom of glucagonoma. NME is presented by erythematous blisters and swelling in areas prone to friction, such as buttocks, groin, lower abdomen, and perineum.
Glucagonoma Treatment
Octreotide is administered to treat the glucagon production. Octreotide is a somatostatin analog and works by decreasing the glucagon release. Other medications include streptozotocin and doxorubicin, which effectively damage certain alpha cells, although they do not cure the tumor. This treatment also slows down the onset of symptoms. A surgical resection is the only known effective and curative therapy for glucagonoma. The procedure completely removes the tumors and even reverses symptoms in some cases.