Definition
Glucagon cell hyperplasia and neoplasia (GCHN) is the histological phenotype of various rare underlying conditions. By pathogenesis it can be divided into reactive, functional, and nonfunctional GCHN.
Reactive GCHN is characterized by germline loss-of-function mutations in the glucagon receptor gene (GCGR) on chromosome 17q25.3, inherited in an autosomal recessive fashion (Mahvash syndrome (Yu et al. 2008)). Hyperaminoacidemia, the result of absent glucagon signaling specifically in the liver, causes pancreatic alpha cell hyperplasia and/or neoplasia, which in turn results in hyperglucagonemia without glucagonoma syndrome due to the defective or absent GCGR (Solloway et al. 2015). Because of this, glucagon-producing pancreatic neuroendocrine tumors (PanNETs) are per definition nonfunctional in reactive GCHN.
Functional and nonfunctional GCHN lack GCGRmutations, and several other genes...
References and Further Reading
Anlauf, M., Schlenger, R., Perren, A., et al. (2006). Microadenomatosis of the endocrine pancreas in patients with and without the multiple endocrine neoplasia type 1 syndrome. The American Journal of Surgical Pathology, 30, 560–574.
Lubensky, I. A., Pack, S., Ault, D., et al. (1998). Multiple neuroendocrine tumors of the pancreas in von Hippel-Lindau disease patients: Histopathological and molecular genetic analysis. The American Journal of Pathology, 153, 223–231.
Lucas, M., Yu, V., & Yu, R. (2013). Mahvash disease: Pancreatic neuroendocrine tumor syndrome caused by inactivating glucagon receptor mutation. Journal of Molecular and Genetic Medicine, 7, 4.
Sipos, B., Sperveslage, J., Anlauf, M., et al. (2015). Glucagon cell hyperplasia and neoplasia with and without glucagon receptor mutations. The Journal of Clinical Endocrinology and Metabolism, 100, E783–E788.
Solloway, M. J., Madjidi, A., Gu, C., et al. (2015). Glucagon couples hepatic amino acid catabolism to mTOR-dependent regulation of alpha-cell mass. Cell Reports, 12, 495–510.
Yu, R., Nissen, N. N., Dhall, D., et al. (2008). Nesidioblastosis and hyperplasia of alpha cells, microglucagonoma, and nonfunctioning islet cell tumor of the pancreas: Review of the literature. Pancreas, 36, 428–431.
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Hackeng, W.M., Luchini, C., Brosens, L.A. (2020). Glucagon Cell Hyperplasia and Neoplasia. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5509-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5509-1
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