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==Overview==
==Overview==
Most [[Adrenocortical carcinoma|adrenocortical carcinomas]] are sporadic, but some occur as a component of hereditary [[cancer]] [[syndromes]] such as [[Li-Fraumeni syndrome]], [[Beckwith-Wiedemann syndrome]], and [[Multiple endocrine neoplasia type 1]]([[MEN1]]). Genetic basis of sporadic incidentaloma includes ''[[TP53 (gene)|TP53]]'' [[gene]]. A role for the ''[[TP53 (gene)|TP53]]'' [[tumor suppressor gene]] in sporadic ACCs is suggested by the frequent finding of [[loss of heterozygosity]] (LOH) at the 17p13 locus in sporadic ACCs. Another [[chromosomal]] [[locus]] that is strongly implicated in the pathogenesis of ACC is 11p, the area of abnormality in [[Beckwith-Wiedemann syndrome]] and the site of the [[Insulin-like growth factor 2|insulin-like growth factor-2]] (IGF-2) [[gene]].
Most [[Adrenocortical carcinoma|adrenocortical carcinomas]] are sporadic, but some occur as a component of hereditary [[cancer]] [[syndromes]] such as [[Li-Fraumeni syndrome]], [[Beckwith-Wiedemann syndrome]], and [[Multiple endocrine neoplasia type 1]]([[MEN1]]).


==Risk Factors==
==Risk Factors==
Most [[Adrenocortical carcinoma|adrenocortical carcinomas]] are sporadic, but some occur as a component of hereditary [[cancer]] [[syndromes]].<ref name="pmid14685087">{{cite journal| author=Sidhu S, Sywak M, Robinson B, Delbridge L| title=Adrenocortical cancer: recent clinical and molecular advances. | journal=Curr Opin Oncol | year= 2004 | volume= 16 | issue= 1 | pages= 13-8 | pmid=14685087 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14685087  }}</ref><ref name="pmid2300390">{{cite journal| author=Lynch HT, Radford B, Lynch JF| title=SBLA syndrome revisited. | journal=Oncology | year= 1990 | volume= 47 | issue= 1 | pages= 75-9 | pmid=2300390 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2300390  }}</ref>
Most [[Adrenocortical carcinoma|adrenocortical carcinomas]] are sporadic, but some occur as a component of hereditary [[cancer]] [[syndromes]].<ref name="pmid14685087">{{cite journal| author=Sidhu S, Sywak M, Robinson B, Delbridge L| title=Adrenocortical cancer: recent clinical and molecular advances. | journal=Curr Opin Oncol | year= 2004 | volume= 16 | issue= 1 | pages= 13-8 | pmid=14685087 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14685087  }}</ref><ref name="pmid2300390">{{cite journal| author=Lynch HT, Radford B, Lynch JF| title=SBLA syndrome revisited. | journal=Oncology | year= 1990 | volume= 47 | issue= 1 | pages= 75-9 | pmid=2300390 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2300390  }}</ref>
* [[Heredity|Hereditary]] [[cancer]] [[syndromes]]:   
* [[Heredity|Hereditary]] [[cancer]] [[syndromes]]:   
** [[Li-Fraumeni syndrome]] (associated with inactivating mutations of the ''[[TP53 (gene)|TP53]]'' [[tumor suppressor gene]] on chromosome 17p):  
** [[Li-Fraumeni syndrome]] (associated with inactivating [[Mutation|mutations]] of the ''[[TP53 (gene)|TP53]]'' [[tumor suppressor gene]] on chromosome 17p):  
***[[Breast cancer]]
***[[Breast cancer]]
*** Soft tissue and [[bone sarcoma]]
*** Soft tissue and [[bone sarcoma]]
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***[[Wilms' tumor]][[Neuroblastoma| Neuroblastoma]]
***[[Wilms' tumor]][[Neuroblastoma| Neuroblastoma]]
***[[Hepatoblastoma]]
***[[Hepatoblastoma]]
**[[Multiple endocrine neoplasia type 1]] ([[MEN1]]) (associated with inactivating mutations of the [[MEN1|''MEN1'' gene]] on chromosome 11q):
**[[Multiple endocrine neoplasia type 1]] ([[MEN1]]) (associated with inactivating mutations of the [[MEN1|''MEN1'' gene]] on [[chromosome]] 11q):
***[[Parathyroid adenoma|Parathyroid adenoma]]
***[[Parathyroid adenoma|Parathyroid adenoma]]
*** [[Pituitary adenoma|Pituitary adenoma]]  
*** [[Pituitary adenoma|Pituitary adenoma]]  

Latest revision as of 16:43, 7 November 2017

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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]

Overview

Most adrenocortical carcinomas are sporadic, but some occur as a component of hereditary cancer syndromes such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and Multiple endocrine neoplasia type 1(MEN1).

Risk Factors

Most adrenocortical carcinomas are sporadic, but some occur as a component of hereditary cancer syndromes.[1][2]

References

  1. Sidhu S, Sywak M, Robinson B, Delbridge L (2004). "Adrenocortical cancer: recent clinical and molecular advances". Curr Opin Oncol. 16 (1): 13–8. PMID 14685087.
  2. Lynch HT, Radford B, Lynch JF (1990). "SBLA syndrome revisited". Oncology. 47 (1): 75–9. PMID 2300390.

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