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Genomic and expression profiling of adrenocortical carcinoma: application to diagnosis, prognosis and treatment

Clinical Translational Research Division, Translational Genomics Research Institute, 445 N. 5th Street, Phoenix, AZ 85004, USA.

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Email the corresponding author at kbussey@tgen.org

Michael J Demeure

† Author for correspondence

Clinical Translational Research Division, Translational Genomics Research Institute, Director, Endocrine Tumors Center, Scottsdale Healthcare, 10460 N. 92nd St, Suite 200, Scottsdale, AZ 85258, USA.

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Email the corresponding author at mdemeure@tgen.org

Published Online:11 Jun 2009https://doi.org/10.2217/fon.09.45

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Abstract

Adrenocortical carcinoma (ACC) is an aggressive endocrine tumor with a poor 5-year survival rate of 10–20%. Current therapy is often ineffective and may be associated with intolerable side effects. Although ACC is extremely rare, recent advances in genomic and expression profiling, coupled with knowledge gained from the study of the inherited syndromes that increase ACC risk, are beginning to bring together a picture of a tumor type dependent on p53, the G2/M cell cycle transition and IGF2 stimulation. Nevertheless, ACC remains a heterogeneous disease. Only recently have sufficient tumors been characterized and results published to permit an exploration of this diversity. Advances in treatment will depend on exploiting those pathways already implicated in ACC, along with those yet to be identified, and testing those treatments in better models of the disease than the three cell lines that currently exist and are widely available to the community.

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Papers of special note have been highlighted as: ▪ of interest ▪▪ of considerable interest

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Published online 11 June 2009

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The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

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