Do glucagonomas always produce glucagon?
- PMID: 26773171
- PMCID: PMC4765933
- DOI: 10.17305/bjbms.2015.794
Do glucagonomas always produce glucagon?
Abstract
Pancreatic islet α-cell tumours that overexpress proglucagon are typically associated with the glucagonoma syndrome, a rare disease entity characterised by necrolytic migratory erythema, impaired glucose tolerance, thromboembolic complications and psychiatric disturbances. Paraneoplastic phenomena associated with enteric overexpression of proglucagon-derived peptides are less well recognized and include gastrointestinal dysfunction and hyperinsulinaemic hypoglycaemia. The diverse clinical manifestations associated with glucagon-expressing tumours can be explained, in part, by the repertoire of tumorally secreted peptides liberated through differential post-translational processing of tumour-derived proglucagon. Proglucagon-expressing tumours may be divided into two broad biochemical subtypes defined by either secretion of glucagon or GLP-1, GLP-2 and the glucagon-containing peptides, glicentin and oxyntomodulin, due to an islet α-cell or enteroendocrine L-cell pattern of proglucagon processing, respectively. In the current review we provide an updated overview of the clinical presentation of proglucagon-expressing tumours in relation to known physiological actions of proglucagon-derived peptides and suggest that detailed biochemical characterisation of the peptide repertoire secreted from these tumours may provide new opportunities for diagnosis and clinical management.
Figures


References
-
- Kaltsas GA, Besser GM, Grossman AB. The Diagnosis and Medical Management of Advanced Neuroendocrine Tumors. Endocrine Reviews. 2004;25(3):458–511. http://dx.doi.org/10.1210/er.2003-0014 . - PubMed
-
- Economopoulos P, Christopoulos C. Glucagonoma. Annals of Gastroenterology. 2007
-
- Öberg K. Neuroendocrine gastro-enteropancreatic tumors - from eminence based to evidence-based medicine - A Scandinavian view. Scandinavian journal of gastroenterology. 2015;50(6):727–39. http://dx.doi.org/10.3109/00365521.2015.1033001 . - PubMed
-
- Yu R, Ren S-G, Mirocha J. Glucagon receptor is required for long-term survival:A natural history study of the Mahvash disease in a murine model. Endocrinología y Nutrición. 2012;59(9):523–30. http://dx.doi.org/10.1016/j.endonu.2012.06.006 . - PubMed
-
- Ito T, Igarashi H, Jensen RT. Pancreatic neuroendocrine tumors:Clinical features, diagnosis and medical treatment:Advances. Best Practice & Research Clinical Gastroenterology. 26(6):737–53. http://dx.doi.org/10.1016/j.bpg.2012.12.003 . - PMC - PubMed
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical