Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center (UTMDACC), Houston, TX.
Department of Medicine, St. Louis University, St. Louis, MO.
Eur J Endocrinol. 2013 Oct 23;169(6):891-899. doi: 10.1530/EJE-13-0519. Print 2013 Dec.
Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Herein, we describe the clinical features and outcomes for a large series of ACC patients.
Retrospective review of ACC patients seen at The University of Texas MD Anderson Cancer Center from 1998 through 2011.
A total of 330 patients with median age at diagnosis of 48.5 years; 12 (3.6%) patients were under 18 years. Hormonally functioning tumors represented 41.8% (n=138) of all cases. Surgical resection for the primary tumor was done in 275 (83.3%) patients (45 at MD Anderson (16.4%)). For those who had surgical resection, the median local-recurrence-free time was 1.04 years. Factors associated with local recurrence included positive surgical margins (P=0.007) and advanced disease stage (P=0.026). Median overall survival time for all patients was 3.21 years. Median survival times were 24.1, 6.08, 3.47, and 0.89 years for stages I, II, III, and IV respectively. In multivariable analysis, older age, functioning tumors, and higher disease stage remained significant prognostic factors associated with poor survival.
ACC prognosis remains poor with the use of currently available treatments. Older age, functioning tumors, and incomplete resections are clinical factors associated with poor survival. Surgical expertise is important to achieve complete resections and to improve outcome.
肾上腺皮质癌（ACC）是一种预后不良的罕见恶性肿瘤。在此，我们描述了一系列 ACC 患者的临床特征和结局。
回顾性分析 1998 年至 2011 年在德克萨斯大学 MD 安德森癌症中心就诊的 ACC 患者。
共 330 例患者，中位诊断年龄为 48.5 岁；12 例（3.6%）患者年龄小于 18 岁。功能性肿瘤占所有病例的 41.8%（n=138）。275 例（83.3%）患者进行了原发性肿瘤切除术（MD 安德森医院 45 例（16.4%））。对于接受手术切除的患者，局部无复发生存时间的中位数为 1.04 年。局部复发的相关因素包括手术切缘阳性（P=0.007）和疾病分期较晚（P=0.026）。所有患者的中位总生存时间为 3.21 年。各分期的中位生存时间分别为 I 期 24.1 年、II 期 6.08 年、III 期 3.47 年和 IV 期 0.89 年。多变量分析显示，年龄较大、功能性肿瘤和较高的疾病分期仍然是与不良生存相关的预后不良的显著预测因素。
尽管采用了目前可用的治疗方法，ACC 的预后仍然很差。年龄较大、功能性肿瘤和不完全切除是与不良生存相关的临床因素。手术专业知识对于实现完全切除和改善结局非常重要。
