Pheochromocytoma risk factors: Difference between revisions

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==Overview==
==Overview==
Pheochromocytoma is more common in people between 40 and 60 years old, men, people with family history of multiple endocrine neoplasia, von Hippel-Lindau disease, neurofibromatosis 1, hereditary paraganglioma syndromes.
The most potent risk factor for pheochromocytoma is a family history of [[Multiple endocrine neoplasia|multiple endocrine neoplasias,]] [[Von Hippel-Lindau disease]], [[Neurofibromatosis type I|neurofibromatosis type 1]], [[Paraganglioma|hereditary paraganglioma syndromes]].  


==Risk Factors==
==Risk Factors==


The most portent risk factors of pheochromocytoma are:
The most potent risk factor in the development of pheochromocytoma is a family history of [[Multiple endocrine neoplasia|multiple endocrine neoplasias,]] [[Von Hippel-Lindau disease]], [[Neurofibromatosis type I|neurofibromatosis type 1]] or [[Paraganglioma|hereditary paraganglioma syndromes]].


=== '''Age:''' ===
===Common Risk Factors===
Occurs in third to fifth decades of life; the average age at diagnosis is 24.9 years in hereditary cases and 43.9 years in sporadic cases.<sup>[[Pheochromocytoma epidemiology and demographics#cite note-cancergov-1|[1]]]</sup>. Hereditary tumors present at a younger age than sporadic.
*Common risk factors in the development of pheochromocytoma include harboring the following genes:
**[[RET gene|RET]] gene ([[MEN, type 2a|MEN 2A]], [[Multiple endocrine neoplasia type 2|MEN 2B]] [[Syndrome|syndromes]])
**[[NF1|NF1 gene]]
**[[Von Hippel-Lindau tumor suppressor|VHL gene]] ([[Von Hippel-Lindau disease|VHL disease]])
**[[SDHD]], [[SDHB]], and [[SDHC]] genes of the [[Mitochondrial|mitochondrial complex]] <ref name="pmid15883706">{{cite journal| author=Gimm O| title=Pheochromocytoma-associated syndromes: genes, proteins and functions of RET, VHL and SDHx. | journal=Fam Cancer | year= 2005 | volume= 4 | issue= 1 | pages= 17-23 | pmid=15883706 | doi=10.1007/s10689-004-5740-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15883706  }} </ref>


=== '''Family history:''' ===
===Less Common Risk Factors===
Ten percent of pheochromocytomas are linked to hereditary causes:
*Less common risk factors in the development of pheochromocytoma include harboring the following genes:
* '''Multiple endocrine neoplasia type 2''' is a hereditary condition associated with medullary thyroid cancer and other types of cancer, including pheochromocytoma.
**[[SDHA]]
{| class="wikitable"
**[[SDHAF2]]
!MEN1
** [[TMEM127]] (transmembrane protein 127)
!MEN2
** [[MAX (gene)|MAX]] (myc-associated factor X)
|-
** [[Fumarate hydratase|FH]] (fumarate hydratase)
|
** [[PDH complex|PDH1]], PDH2 (pyruvate dehydrogenase)
* Medullary thyroid cancer,
** [[Hypoxia inducible factors|HIF1alpha]] (hypoxia-inducible factor)
* Pheochromocytoma
** [[MDH1|MDH2]] (malate dehydrogenase)
* Primary hyperparathyroidism
** KIF1Bß (kinesin family member) genes. <ref>{{cite book | last = Jameson | first = J | title = Harrison's Principles of Internal Medicine 19th Edition and Harrison's Manual of Medicine 19th Edition VAL PAK | publisher = McGraw-Hill Medical | location = New York | year = 2017 | isbn = 978-1260128857 }} </ref>
|
* Medullary thyroid cancer
* Pheochromocytoma
* Mucosal neuromas
* Marfanoid habitus
|}
* '''Von Hippel-Lindau disease''' can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
* '''Neurofibromatosis 1 (NF1)''' results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
* '''Hereditary paraganglioma syndromes''' are inherited disorders that result in either pheochromocytomas or paragangliomas.


==References==
==References==
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Latest revision as of 00:32, 25 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D[2] Ifrah Fatima, M.B.B.S[3]

Overview

The most potent risk factor for pheochromocytoma is a family history of multiple endocrine neoplasias, Von Hippel-Lindau disease, neurofibromatosis type 1, hereditary paraganglioma syndromes.

Risk Factors

The most potent risk factor in the development of pheochromocytoma is a family history of multiple endocrine neoplasias, Von Hippel-Lindau disease, neurofibromatosis type 1 or hereditary paraganglioma syndromes.

Common Risk Factors

Less Common Risk Factors

  • Less common risk factors in the development of pheochromocytoma include harboring the following genes:
    • SDHA
    • SDHAF2
    • TMEM127 (transmembrane protein 127)
    • MAX (myc-associated factor X)
    • FH (fumarate hydratase)
    • PDH1, PDH2 (pyruvate dehydrogenase)
    • HIF1alpha (hypoxia-inducible factor)
    • MDH2 (malate dehydrogenase)
    • KIF1Bß (kinesin family member) genes. [2]

References

  1. Gimm O (2005). "Pheochromocytoma-associated syndromes: genes, proteins and functions of RET, VHL and SDHx". Fam Cancer. 4 (1): 17–23. doi:10.1007/s10689-004-5740-1. PMID 15883706.
  2. Jameson, J (2017). Harrison's Principles of Internal Medicine 19th Edition and Harrison's Manual of Medicine 19th Edition VAL PAK. New York: McGraw-Hill Medical. ISBN 978-1260128857.