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Tumors of the Central Nervous System, Volume 10 pp 71–83Cite as

Pituitary Tumors: Genetics and Heritable Predisposition

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Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 10))

Abstract

Pituitary tumors are the most common intracranial tumors. Most pituitary tumors are thought to be sporadic, with estimates showing that genetic heritability in the form of traditional syndromes, including multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC), accounts for only 5% of all cases. The monoclonality of pituitary tumors is a widely established model in which a genetic mutation in one cell leads to the formation of an adenoma. On a larger scale, however, the pituitary gland may contain multiple hyperplastic cells, each with its own origin. The predominant cell type within the adenoma is dependent on a variety of oncogenes and tumor suppressor genes including GSP, RAS, Cyclin D1, PTTG, and p53. Multiple other studies show germline mutations in a variety of additional genes, including AIP, BMP-4, CDKN1B, CDKN2A, CDKN2C, GADD45G, PDt-FGFR4, PKC, PRKAR1A, RB, WIF1, and ZAC. More recently, studies of genetic mutations leading to pituitary adenomas and population studies of patients and families with pituitary adenomas have revealed a significant heritable predisposition for pituitary tumors outside of traditional syndromes. These studies, while confirming the heritability of pituitary tumors, unfortunately only provide a glimpse into the multifactorial cause of pituitary adenomas.

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Authors and Affiliations

  1. Department of Neurosurgery, University of Utah, Clinical Neurosciences Center, 175 North Medical Drive East, Salt Lake City, UT, 84132, USA

    Ricky R. Kalra, Philipp Taussky, Toba Niazi & William Couldwell

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  1. Ricky R. Kalra
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  2. Philipp Taussky
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  3. Toba Niazi
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  4. William Couldwell
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Correspondence to William Couldwell .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Kalra, R.R., Taussky, P., Niazi, T., Couldwell, W. (2013). Pituitary Tumors: Genetics and Heritable Predisposition. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 10. Tumors of the Central Nervous System, vol 10. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5681-6_8

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  • DOI: https://doi.org/10.1007/978-94-007-5681-6_8

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