- Type of functional pancreatic neuroendocrine neoplasm (pNEN)
- Accounts for 2% of islet cell carcinomas
Glucagonomas are neuroendocrine tumors of the pancreatic islets that secrete glucagon.
The first case was described in 1942 by Becker et al. in a 45-year-old woman presenting with widespread dermatitis, weight loss, glossitis, and abnormal glucose tolerance associated with an islet cell neoplasm of the pancreas on autopsy specimen. In 1966, McGavran and colleagues identified the classic symptoms of mild diabetes and dermatitis along with elevated glucagon levels related to a metastatic alpha cell tumor of the pancreatic islets.
Glucagonoma syndromeGlucagonoma syndrome occurs due to the effects of elevated glucagon levels secreted by the tumor.
Classical description of glucagonoma syndrome includes the 4D syndrome, consisting of dermatosis (necrolytic migratory erythema), new onset of diabetes mellitus, deep vein thrombosis, and depression along with anemia, glossitis, weight loss in the presence of hyperglucagonemia
Necrolytic migratory erythema (NME) (~65–70% cases)hallmark of glucagonoma syndrome and is characterized by an annular pattern of erythema with centrally formed fragile vesicles, bullae and crusts
Diabetes mellitus (40% on presentation but 90% develop over time)Clinical hallmark in which elevated glucagon levels promote the glucose output and antagonize the effect of insulin.
- Depression (50% cases)
- Other features: Dementia, psychosis, agitation, hyperreflexia, ataxia, paranoid delusions, and proximal muscle weakness
- Weight loss (60% cases)
- Anemia (50% cases)
- Deep vein thrombosis (DVT) (50% cases)
- Glossitis/stomatitis/cheilitis (40% cases)
- Chronic diarrhea (30% cases)
- Pseudoglucagonoma syndrome: Celiac disease, malabsorption, cirrhosis, malignancy and pancreatitis may also present NME-like skin lesions
Surgical management:Only method of treatment.
Non-surgical mangement:Temporary relief of symptoms
- Somatostatin analogues (SSA)
- Amino acid solution infusion
- Transarterial chemoembolization
- Radiation therapy
- Peptide receptor radioligand therapy