The Oncologist, Vol. 13, No. 5, 548-561, May 2008; doi:10.1634/theoncologist.2007-0243 © 2008 AlphaMed Press
Molecular Markers and the Pathogenesis of Adrenocortical CanceraCancer Genetics, Kolling Institute of Medical Research, University of Sydney, bDepartment of Surgery, Bankstown Hospital and University of New South Wales, cDepartment of Endocrinology, and dDepartment of Endocrine and Oncology Surgery, Royal North Shore Hospital, St. Leonards, Australia and University of Sydney Key Words. Adrenocortical carcinoma • Adrenocortical adenoma • Adrenocortical tumors • Molecular marker Correspondence: Stan Sidhu, MBBS, PhD, FRACS, Department of Endocrine and Oncology Surgery, Royal North Shore Hospital, St. Leonards, NSW 2065, Australia. Telephone: 61-2-94371731; Fax: 61-2-99268523; e-mail: stansidhu{at}nebsc.com.au Received December 12, 2007; accepted for publication March 24, 2008. Disclosure: No potential conflicts of interest were reported by the authors.
Adrenal tumors are common, with an estimated incidence of 7.3% in autopsy cases, while adrenocortical carcinomas (ACCs) are rare, with an estimated prevalence of 4–12 per million population. Because the prognoses for adrenocortical adenomas (ACAs) and ACCs are vastly different, it is important to be able to accurately differentiate the two tumor types. Advancement in the understanding of the pathophysiology of ACCs is essential for the development of more sensitive means of diagnosis and treatment, resulting in better clinical outcome. Adrenocortical tumors (ACTs) occur as a component of several hereditary tumor syndromes, which include the Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia 1, Carney complex, and congenital adrenal hyperplasia. The genes involved in these syndromes have also been shown to play a role in the pathogenesis of sporadic ACTs. The adrenocorticotropic hormone–cAMP–protein kinase A and Wnt pathways are also implicated in adrenocortical tumorigenesis. The aim of this review is to summarize the current knowledge on the molecular mechanisms involved in adrenocortical tumorigenesis, including results of comparative genomic hybridization, loss of heterozygosity, and microarray gene-expression profiling studies.
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