title: Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants.
abstract: Adrenocortical carcinoma (ACC) is a rare, heterogeneous malignancy with a poor prognosis. According to WHO classification 2004, ACC variants include oncocytic ACCs, myxoid ACCs and ACCs with sarcomatous areas. Herein, we provide a comprehensive review of these rare subtypes of adrenocortical malignancy and emphasize their clinicopathological features with the aim of elucidating aspects of diagnostic categorization, differential diagnostics and biological behavior. The issue of current terminology, applied to biphasic tumors with pleomorphic, sarcomatous or sarcomatoid elements arising in adrenal cortex, is also discussed. We additionally present emerging evidence concerning the adrenal cortical tumorigenesis and the putative adenoma-carcinoma sequence as well.
authors.affiliation: Department of Pathology, Josephine Nefkens Institute, Erasmus MC-University Medical Center Rotterdam, Rotterdam, The Netherlands. r.dekrijger@erasmusmc.nl
authors.forename: Ronald R
authors.initials: RR
authors.lastname: de Krijger
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Papathomas
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000145:classification* / Q000175:diagnosis / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000145:classification* / Q000175:diagnosis / Q000473:pathology*; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D009626:Terminology as Topic; D014944:World Health Organization
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