Division of Endocrinology and Metabolism, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.
Faculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan.
Front Endocrinol (Lausanne). 2023 Mar 10;14:1134643. doi: 10.3389/fendo.2023.1134643. eCollection 2023.
The prognosis of adrenocortical carcinoma (ACC) is poor but highly variable. The present study aimed to characterize patients with ACC at a single center in Taiwan and to determine the prognostic predictors of overall and progression-free survival.
Medical records of patients, who were diagnosed with ACC at Taipei Veterans General Hospital between January 1992 and June 2021, were reviewed. Patient demographics, tumor characteristics, and subsequent treatment were analyzed with regard to overall survival and progression-free survival using Kaplan-Meier methods and a Cox regression model.
Sixty-seven patients were included. Females (65.7%) were more susceptible to ACC, with a younger onset and active hormonal secretion. One-half of the patients exhibited distant metastases at the time of diagnosis. The European Network for the Study of Adrenal Tumours (ENSAT) stage (hazard ratio [HR] 3.60 [95% confidence interval (CI) 1.25-10.38]; p=0.018), large vessel invasion (HR 5.19 [95% CI 1.75-15.37]; p=0.003), and mitotane use (HR 0.27 [95% CI 0.11-0.70]; p=0.007) were significantly associated with overall survival (OS). There was no single factor independently associated with progression-free survival.
ENSAT stage had a substantial impact on overall survival though there was no difference in OS between patients with stage II and stage III ACC. Large vessel invasion portended poor prognosis and influenced OS significantly. Moreover, mitotane only improved clinical outcomes of patients with stage IV disease.
肾上腺皮质癌（adrenocortical carcinoma，ACC）的预后较差，但具有高度可变性。本研究旨在描述台湾某单一中心的 ACC 患者，并确定总生存和无进展生存的预后预测因素。
回顾性分析 1992 年 1 月至 2021 年 6 月在台北荣民总医院诊断为 ACC 的患者的病历。采用 Kaplan-Meier 方法和 Cox 回归模型，根据总生存和无进展生存，分析患者的人口统计学特征、肿瘤特征和后续治疗。
共纳入 67 例患者。女性（65.7%）更易患 ACC，发病年龄更小，且有活跃的激素分泌。一半的患者在诊断时已发生远处转移。欧洲肾上腺肿瘤研究网络（ENSAT）分期（风险比[HR]3.60[95%置信区间（CI）1.25-10.38]；p=0.018）、大血管侵犯（HR 5.19[95%CI 1.75-15.37]；p=0.003）和米托坦治疗（HR 0.27[95%CI 0.11-0.70]；p=0.007）与总生存（OS）显著相关。无单一因素与无进展生存独立相关。
ENSAT 分期对总生存有显著影响，但 II 期和 III 期 ACC 患者的 OS 无差异。大血管侵犯预示预后不良，对 OS 有显著影响。此外，米托坦仅改善了 IV 期疾病患者的临床结局。
