Glucagonoma is a very rare tumor of the pancreatic alpha cells that results in the overproduction of the hormone, glucagon.
A neuroendocrine tumor of the pancreatic islets that secrete glucagon.
It is associated with a rash called necrolytic migratory erythema, weight loss, and mild diabetes mellitus.
Most cases are contracted spontaneously.
However, about 10% of cases are associated with multiple endocrine neoplasia type 1 (MEN-1) syndrome.
A family history of multiple endocrine neoplasia type 1 (MEN1) is a risk factor.
Glucagonoma results from the overproduction of glucagon, a peptide hormone located in the pancreatic alpha cells.
Classic symptoms and findings include: necrolytic migratory erythema (NME), diabetes mellitus, and weight loss.
NME is present in about 70% of cases.
NME is characterized by erythematous lesions over the distal extremities and the groin area.
NME has occasionally been seen in people who do not have glucagonoma.
Weight loss, the most commonly associated effect with glucagonoma, results from the glucagon hormone, which prevents the uptake of glucose by somatic cells.
Diabetes is not present in all cases of glucagonoma, but does frequently result from the insulin and glucagon imbalance.
The presence of glucagonoma syndrome, the symptoms that accompany the pancreatic tumor, as well as elevated levels of glucagon in the blood, are what is used to diagnose glucagonoma.
About 60% of people diagnosed with glucagonoma are women.
Most of those that are diagnosed are between 45–60 years of age.
Hyperglycemia and Weight loss or a common findings.
The metastatic tumor is resistant to chemotherapy.
The only curative therapy for glucagonoma is surgery, and even this is not always successful.
Elevated glucagon secretion can be treated with the administration of octreotide, a somatostatin analog, which inhibits the release of glucagon.
Doxorubicin and streptozotocin have also been used successfully to selectively damage alpha cells of the pancreatic islets.
There are fewer than one in 20 million worldwide.
Glucagonoma accounts for approximately 1% of neuroendocrine tumors, although this may be an underestimate given that glucagonoma is associated with non-specific symptoms.