Department of Internal Medicine I, Endocrine Unit, University Hospital, University of Würzburg, Oberdürrbacher Straße 6, 97080 Würzburg, Germany.
Nat Rev Endocrinol. 2011 Jun;7(6):323-35. doi: 10.1038/nrendo.2010.235. Epub 2011 Mar 8.
Adrenocortical carcinoma is a rare heterogeneous neoplasm with an incompletely understood pathogenesis and a poor prognosis. Previous studies have identified overexpression of insulin-like growth factor 2 (IGF-2) and constitutive activation of β-catenin as key factors involved in the development of adrenocortical carcinoma. Most patients present with steroid hormone excess, for example Cushing syndrome or virilization, or abdominal mass effects, but a growing proportion of patients with adrenocortical carcinoma (currently >15%) is initially diagnosed incidentally. No general consensus on the diagnostic and therapeutic measures for adrenocortical carcinoma exists, but collaborative efforts, such as international conferences and networks, including the European Network for the Study of Adrenal Tumors (ENSAT), have substantially advanced the field. In patients with suspected adrenocortical carcinoma, a thorough endocrine and imaging work-up is recommended to guide the surgical approach aimed at complete resection of the tumor. To establish an adequate basis for treatment decisions, pathology reports include the Weiss score to assess malignancy, the resection status and the Ki67 index. As recurrence is frequent, close follow-up initially every 3 months is mandatory. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Results of a large phase III trial in advanced adrenocortical carcinoma are anticipated for 2011 and will hopefully establish a benchmark therapy. New targeted therapies, for example, IGF-1 receptor inhibitors, are under investigation and may soon improve current treatment options.
肾上腺皮质癌是一种罕见的异质性肿瘤，其发病机制尚未完全阐明，预后较差。先前的研究已经确定胰岛素样生长因子 2（IGF-2）的过表达和β-连环蛋白的组成性激活是参与肾上腺皮质癌发生的关键因素。大多数患者表现为类固醇激素过多，例如库欣综合征或男性化，或腹部肿块效应，但越来越多的肾上腺皮质癌（目前>15%）患者最初是偶然诊断出来的。目前尚无关于肾上腺皮质癌的诊断和治疗措施的普遍共识，但合作努力，如国际会议和网络，包括欧洲肾上腺肿瘤研究网络（ENSAT），已经大大推动了该领域的发展。对于疑似肾上腺皮质癌的患者，建议进行全面的内分泌和影像学检查，以指导旨在完全切除肿瘤的手术方法。为了为治疗决策提供充分的依据，病理报告包括评估恶性程度的 Weiss 评分、切除状态和 Ki67 指数。由于复发频繁，最初必须每 3 个月密切随访一次。大多数患者受益于辅助米托坦治疗。对于转移性疾病，米托坦是初始治疗的基石，如果病情进展，应添加细胞毒药物。预计 2011 年将进行一项针对晚期肾上腺皮质癌的大型 III 期试验，希望该试验将为治疗建立一个基准。新的靶向治疗方法，例如 IGF-1 受体抑制剂，正在研究中，可能很快会改善当前的治疗选择。
