Adrenocortical carcinoma (ACC) is a rare yet highly malignant tumor associated with significant morbidity and mortality. This study aims to delineate the clinical features, survival patterns, and treatment modalities of ACC, providing insights into the disease's prognosis. A retrospective analysis of 157 ACC patients was performed to assess treatment methodologies, demographic patterns, pathological and clinical attributes, and laboratory results. The data were extracted from the hospital's database. Survival analyses were conducted using the Kaplan–Meier method, with univariate and multivariate analyses being performed through the log-rank test and Cox regression analyses. The median age was 45, and 89.4% had symptoms at the time of diagnosis. The median tumor size was 12 cm. A total of 117 (79.6%) patients underwent surgery. A positive surgical border was detected in 26 (24.1%) patients. Adjuvant therapy was administered to 44.4% of patients. The median overall survival for the entire cohort was 44.3 months. Median OS was found to be 87.3 months (95% confidence interval [CI] 74.4-100.2) in stage 2, 25.8 (95% CI 6.5-45.1) months in stage 3, and 13.3 (95% CI 7.0-19.6) months in stage 4 disease. Cox regression analysis identified age, Ki67 value, Eastern Cooperative Oncology Group performance status, and hormonal activity as significant factors associated with survival in patients with nonmetastatic disease. In metastatic disease, only patients who underwent surgery exhibited significantly improved overall survival in univariate analyses. ACC is an uncommon tumor with a generally poor prognosis. Understanding the defining prognostic factors in both localized and metastatic diseases is vital. This study underscores age, Ki67 value, Eastern Cooperative Oncology Group performance status, and hormonal activity as key prognostic determinants for localized disease, offering critical insights into the complexities of ACC management and potential avenues for targeted therapeutic interventions.

中文翻译：

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现实世界中的肾上腺皮质癌：土耳其肿瘤学组 (TOG) 临床特征和治疗的综合分析

肾上腺皮质癌（ACC）是一种罕见但高度恶性的肿瘤，具有显着的发病率和死亡率。本研究旨在描述 ACC 的临床特征、生存模式和治疗方式，为该疾病的预后提供见解。对 157 名 ACC 患者进行回顾性分析，以评估治疗方法、人口统计模式、病理和临床特征以及实验室结果。数据是从医院的数据库中提取的。使用Kaplan-Meier方法进行生存分析，通过对数秩检验和Cox回归分析进行单变量和多变量分析。中位年龄为 45 岁，89.4% 的人在诊断时有症状。肿瘤大小中位数为 12 厘米。共有 117 例（79.6%）患者接受了手术。 26 名 (24.1%) 患者检测到阳性手术边界。 44.4% 的患者接受了辅助治疗。整个队列的中位总生存期为 44.3 个月。第 2 阶段的中位 OS 为 87.3 个月（95% 置信区间 [CI] 74.4-100.2），第 3 阶段为 25.8 (95% CI 6.5-45.1) 个月，第 3 阶段为 13.3 (95% CI 7.0-19.6) 个月。第四阶段疾病。 Cox 回归分析确定年龄、Ki67 值、东部肿瘤合作组表现状态和激素活性是与非转移性疾病患者生存相关的重要因素。在转移性疾病中，只有接受手术的患者在单变量分析中表现出总生存率显着提高。 ACC 是一种罕见的肿瘤，预后通常较差。了解局部疾病和转移性疾病的决定性预后因素至关重要。这项研究强调年龄、Ki67 值、东部肿瘤合作组表现状态和激素活性作为局部疾病的关键预后决定因素，为 ACC 管理的复杂性和针对性治疗干预的潜在途径提供了重要见解。