glucagonoma
Pronunciation: gloo-KAG-oh-noh-muh
A rare functional pancreatic neuroendocrine tumor that secretes excessive amounts of glucagon hormone.
Full Definition
Glucagonomas are rare functional pancreatic neuroendocrine tumors that arise from alpha cells in the pancreatic islets and secrete excessive glucagon. The classic presentation includes the glucagonoma syndrome, characterized by necrolytic migratory erythema (a distinctive skin rash), diabetes mellitus, weight loss, anemia, and thromboembolism. Most glucagonomas are malignant at presentation, with liver metastases commonly present. Diagnosis is confirmed by elevated plasma glucagon levels, typically exceeding 1000 pg/mL. Treatment involves surgical resection when feasible, with somatostatin analogs used for symptom control and systemic therapies for metastatic disease.
Usage
Usage note: Always associated with glucagonoma syndrome when functional; distinguish from other functional PanNETs by hormone profile.
In Context
- "The patient's necrolytic migratory erythema resolved following resection of the glucagonoma." — Endocrinology follow-up note
- "Plasma glucagon levels of 2400 pg/mL supported the diagnosis of glucagonoma syndrome." — Laboratory interpretation