Department of Pathology, Josephine Nefkens Institute, Erasmus MC-University Medical Center Rotterdam, Rotterdam, The Netherlands.
Virchows Arch. 2012 Jan;460(1):9-18. doi: 10.1007/s00428-011-1166-y. Epub 2011 Nov 16.
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.
肾上腺皮质癌（adrenocortical carcinoma，ACC）是一种罕见的、异质性的恶性肿瘤，预后不良。根据 2004 年世界卫生组织（World Health Organization，WHO）分类，ACC 的变异型包括嗜酸细胞瘤、黏液样 ACC 和伴有肉瘤样区域的 ACC。在此，我们全面回顾了这些罕见的肾上腺皮质恶性肿瘤亚型，并强调了它们的临床病理特征，旨在阐明诊断分类、鉴别诊断和生物学行为的各个方面。本文还讨论了目前应用于在肾上腺皮质中发生的具有多形性、肉瘤样或肉瘤样成分的双相肿瘤的术语问题。我们还提供了关于肾上腺皮质肿瘤发生和推测的腺瘤-癌序列的新证据。
