Incidentaloma risk factors
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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). Genetic basis of sporadic incidentaloma includes TP53 gene. A role for the 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 (IGF-2) gene.
Risk Factors
Most adrenocortical carcinomas are sporadic, but some occur as a component of hereditary cancer syndromes.[1][2]
- Hereditary cancer syndromes:
- Li-Fraumeni syndrome (associated with inactivating mutations of the TP53 tumor suppressor gene on chromosome 17p):
- Breast cancer
- Soft tissue and bone sarcoma
- Brain tumors
- Beckwith-Wiedemann syndrome (associated with abnormalities in 11p15):
- Multiple endocrine neoplasia type 1 (MEN1) (associated with inactivating mutations of the MEN1 gene on chromosome 11q):
- Li-Fraumeni syndrome (associated with inactivating mutations of the TP53 tumor suppressor gene on chromosome 17p):
References
- ↑ 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.
- ↑ Lynch HT, Radford B, Lynch JF (1990). "SBLA syndrome revisited". Oncology. 47 (1): 75–9. PMID 2300390.