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Jonathan R. Strosberg, Gary D. Hammer and Gerard M. Doherty

Edited by Kerrin G. Robinson

Adrenocortical carcinomas (ACCs) are rare tumors that arise from the cortex of the adrenal gland with an incidence 1 to 2 per million. The rarity of this tumor translates into a paucity of experience in managing patients in most medical centers. Because clinical series are small and prospective evaluation of treatment strategies is limited, the current state of knowledge is strongly influenced by expert consensus opinion from a few medical centers specializing in ACCs. This article describes the basic diagnostic and prognostic issues in adrenal cancer management, and presents detailed rationales for therapeutic management.