肾上腺皮质癌临床特征及预后分析（附22例）

目的:总结分析肾上腺皮质癌（adrenal cortical carcinoma,ACC）的临床病理特征及治疗情况，寻找其预后预测因子。方法:回顾性分析2010年1月至2018年12月于西安交通大学第一附属医院收治的22例ACC患者的临床病理资料及治疗、预后情况。结果:22例患者平均年龄为（50.59±10.62）岁（27~73岁），其中男性9例、女性13例。19例患者接受手术治疗，21例患者接受辅助性治疗。相较于未发生远处转移的患者，发生远处转移的患者临床分期晚(III期及IV期)、Ki-67指数高（≥10%），且具有统计学差异（P=0.001及P=0.018）。利用Kaplan-Meier生存曲线评估预后情况:接受肾上腺肿瘤切除术的患者远期预后显著优于未接受手术者（P＝0.006）。对于接受NCCN指南推荐治疗方案的患者，II期-III期ACC患者中位生存期显著优于IV期患者（P＜0.001）；而Ki-67指数＜20%的患者中位生存期优于Ki-67指数≥20%的患者（P=0.011）。肿瘤体积、是否存在钙化等特征与预后无关。单因素及多因素COX分析发现临床分期晚（P=0.006）和高Ki-67指数（≥20%,P=0.025）是不良预后的独立危险因素。结论:ACC恶性程度高，病情进展快，完整切除病灶可提高生存率。IV期与高Ki-67指数（≥20%）是不良预后的独立危险因素。

Adrenocortical carcinoma:Clinical features,treatment and prognosis (22 cases)

Objective: To summarize and analyze the clinical features,treatment and prognosis of adrenocortical carcinoma (ACC).Methods:The clinicopathological features,treatment and prognosis of 22 ACC patients admitted to the First Affiliated Hospital of Xi'an Jiaotong University from January 2010 to December 2018 were retrospectively analyzed.The Kaplan-Meier curve was used for overall survival.Results:The average age of 22 patients (9 males and 13 females) was (50.59±10.62) years old （range 27~73 years old）.Nineteen patients received surgery and 21 received adjuvant therapy.Compared with patients without distant metastasis,patients with distant metastasis had later clinical stage (III stage and IV stage,P=0.001) and higher Ki-67 index （≥10%,P=0.018）.Patients receiving adrenal tumor resection had a significantly better long-term prognosis.For patients receiving standardized treatment,clinical stage and Ki-67 index were the main factors affecting prognosis (all P＜0.05).Tumor size and calcification were not correlated with long-term prognosis (P＞0.05).Univariate and multivariate COX analysis showed that advanced stage disease （IV stage） and high Ki-67 index （≥20%） were independent risk factors for poor prognosis （P=0.006 and P=0.025 respectively）.Conclusion:ACC is highly aggressive.Complete resection of the lesion can improve the overall survival.Advanced stage （IV stage） and high Ki-67 index （≥20%） were independent risk factors for poor prognosis.