Departments of Medical Sciences.
Division, University of Turin, Oncology, Pathology Turin, Italy.
Adv Anat Pathol. 2023 Jan 1;30(1):34-46. doi: 10.1097/PAP.0000000000000363. Epub 2022 Sep 8.
Adrenal cortical carcinoma (ACC) is a rare and aggressive malignancy that poses challenging issues regarding the diagnostic workup. Indeed, no presurgical technique or clinical parameters can reliably distinguish between adrenal cortical adenomas, which are more frequent and have a favorable outcome, and ACC, and the final diagnosis largely relies on histopathologic analysis of the surgical specimen. However, even the pathologic assessment of malignancy in an adrenal cortical lesion is not straightforward and requires a combined evaluation of multiple histopathologic features. Starting from the Weiss score, which was developed in 1984, several histopathologic scoring systems have been designed to tackle the difficulties of ACC diagnosis. Dealing with specific histopathologic variants (eg, Liss-Weiss-Bisceglia scoring system for oncocytic ACC) or patient characteristics (eg, Wieneke index in the pediatric setting), these scores remarkably improved the diagnostic workup of ACC and its subtypes. Nevertheless, cases with misleading features or discordant correlations between pathologic findings and clinical behavior still occur. Owing to multicentric collaborative studies integrating morphologic features with ancillary immunohistochemical markers and molecular analysis, ACC has eventually emerged as a multifaceted, heterogenous malignancy, and, while innovative and promising approaches are currently being tested, the future clinical management of patients with ACC will mainly rely on personalized medicine and target-therapy protocols. At the dawn of the new Fifth World Health Organization classification of endocrine tumors, this review will tackle ACC from the pathologist's perspective, thus focusing on the main available diagnostic, prognostic, and predictive tissue-tethered features and biomarkers and providing relevant clinical and molecular correlates.
肾上腺皮质癌 (ACC) 是一种罕见且侵袭性的恶性肿瘤，在诊断方面存在挑战。事实上，没有任何术前技术或临床参数可以可靠地区分更常见且预后良好的肾上腺皮质腺瘤和 ACC，最终诊断主要依赖于手术标本的组织病理学分析。然而，即使是对肾上腺皮质病变恶性程度的病理评估也并不简单，需要综合评估多个组织病理学特征。从 1984 年开发的 Weiss 评分开始，已经设计了几种组织病理学评分系统来解决 ACC 诊断的难题。针对特定的组织病理学变异（例如，用于嗜酸细胞瘤性 ACC 的 Liss-Weiss-Bisceglia 评分系统）或患者特征（例如，儿科中的 Wieneke 指数），这些评分系统显著改善了 ACC 及其亚型的诊断工作流程。然而，仍然存在具有误导性特征或病理发现与临床行为之间存在不一致相关性的病例。由于多中心合作研究将形态学特征与辅助免疫组织化学标志物和分子分析相结合，ACC 最终被认为是一种具有多面性和异质性的恶性肿瘤，虽然目前正在测试创新和有前途的方法，但 ACC 患者的未来临床管理将主要依赖于个性化医学和靶向治疗方案。在新的第五版世界卫生组织内分泌肿瘤分类的黎明时期，本文将从病理学家的角度探讨 ACC，重点介绍主要的可用诊断、预后和预测性组织相关特征和生物标志物，并提供相关的临床和分子相关性。
