Glucagonoma risk factors
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2] Mohammed Abdelwahed M.D[3]
Overview
The most common risk factor in the development of glucagonoma is a positive family history of multiple endocrine neoplasia type1 which is characterized by the presence of pituitary adenomas, islet cell tumors of the pancreas, and hyperparathyroidism.
Risk Factors
- The most common risk factor in the development of glucagonoma is a positive family history of multiple endocrine neoplasia type 1.[1][2]
- It is an autosomal dominant syndrome that is usually caused by mutations in the MEN1 gene.
- It is characterized by the development of the following tumors:[1]
- Pituitary adenomas
- Islet cell tumors of the pancreas (commonly gastrinoma and glucagonoma)
- Parathyroid hyperplasia with resulting hyperparathyroidism
- The gene locus causing multiple endocrine neoplasia type 1 has been localized to chromosome 11q13 by studies of loss of heterozygosity on multiple endocrine neoplasia type 1-associated tumors and by linkage analysis in multiple endocrine neoplasia type 1 families.[3]
- MEN1 spans about 10 Kb and consists of ten exons encoding a 610 amino acid nuclear protein, named menin.[4]
- MEN1 gene is a tumor suppressor gene and causes type 1 multiple endocrine neoplasia by the two hits model for tumor development.[5]
References
- ↑ Afsharfard A, Atqiaee K, Lotfollahzadeh S, Alborzi M, Derakhshanfar A (2012). "Necrolytic migratory erythema as the first manifestation of glucagonoma". Case Rep Surg. 2012: 974210. doi:10.1155/2012/974210. PMC 3434377. PMID 22970401.
- ↑ Glucagonoma. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000326.htm
- ↑ Larsson C, Skogseid B, Oberg K, Nakamura Y, Nordenskjöld M (1988). "Multiple endocrine neoplasia type 1 gene maps to chromosome 11 and is lost in insulinoma". Nature. 332 (6159): 85–7. doi:10.1038/332085a0. PMID 2894610.
- ↑ Byström C, Larsson C, Blomberg C, Sandelin K, Falkmer U, Skogseid B; et al. (1990). "Localization of the MEN1 gene to a small region within chromosome 11q13 by deletion mapping in tumors". Proc Natl Acad Sci U S A. 87 (5): 1968–72. PMC 53606. PMID 1968641.
- ↑ Friedman E, Sakaguchi K, Bale AE, Falchetti A, Streeten E, Zimering MB; et al. (1989). "Clonality of parathyroid tumors in familial multiple endocrine neoplasia type 1". N Engl J Med. 321 (4): 213–8. doi:10.1056/NEJM198907273210402. PMID 2568586.