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Pathogenesis of adrenocortical cancer
Best Practice & Research Clinical Endocrinology & Metabolism, 06/19/09
Bertherat J et al. – Review summarizes the advances in understanding the molecular mechanisms underlying adrenocortical tumour (ACT) development that have been made in the last decade and came from studies of rare familial diseases predisposing to adrenocortical cancer (ACC) and from gene profiling studies of ACT. Review concludes that:
- The role of genetic alterations seems central in the development of ACC.
- Some molecular alterations, such as 17p13 LOH or 11p15 UPD (unipaternal disomy), are common in ACC and might occur early in tumour development.
- Other events, such as TP53 somatic mutations, might be restricted to a subset of aggressive ACCs.
- Considering the major differences in the transcriptome of benign and malignant tumours, it is plausible that ACC and benign adenomas arise from different mechanisms.
- Development of new molecular tools for the assessment of the prognosis of adrenocortical cancer.
- Identification of genes involved in the main chromosomal alterations of adrenocortical cancer (i.e. 17p13, 11q13…)
- Development of therapy targeting the major signalling pathways activated in adrenocortical cancer (i.e. IGF-II, Wnt/β-catenin…)
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