伴皮质醇增多症的肾上腺皮质癌临床特征及预后影响因素分析

Clinical features and prognostic factors of adrenocortical carcinoma with hypercortisolism

目的明确伴皮质醇增多症的肾上腺皮质癌(adrenocortical carcinoma,ACC)患者的临床及病理特征,进一步分析ACC患者生存预后影响因素。方法回顾性收集2003年1月至2022年12月在南京鼓楼医院确诊为ACC患者的临床资料。分析比较伴皮质醇增多症及无功能ACC患者的临床病理特征。采用Kaplan-Meier法做生存分析,采用Cox回归分析ACC患者的预后影响因素。结果61例ACC患者平均年龄(49.33±16.32)岁,中位随访时间为77个月(95%CI 47.49~106.51个月),中位总体生存时间50个月(95%CI 20.44~79.56个月),中位无进展生存时间29个月(95%CI 22.87~35.13个月)。36例患者完善内分泌功能评估,其中伴皮质醇增多症19例(52.77%)。临床病理特征中,伴皮质醇增多症ACC患者低钾血症(42.11%对6.25%,P=0.022)、包膜侵犯(68.42%对25.00%,P=0.018)及远处转移(73.68%对25.00%,P=0.007)的比例显著高于无功能ACC。伴皮质醇增多ACC患者的中位总体生存时间、中位无进展生存时间,显著低于无功能ACC组(总体生存时间39个月对67个月,P=0.009;无进展生存时间30个月对51个月,P=0.040)。多因素Cox回归分析显示,Ki67指数(HR=1.078,P=0.024)是患者死亡的独立危险因素。皮质醇增多症(HR=71.112,P=0.006)、Ki67指数(HR=1.345,P=0.003)、接受辅助治疗(HR=176.652,P=0.012)和手术治疗(HR=0.020,P=0.003)是影响疾病进展的独立危险因素。结论伴皮质醇增多症的ACC更易出现侵犯和远处转移,患者生存时间更短。皮质醇过度分泌是影响ACC患者预后的独立危险因素。

Objective To identify the clinical and pathological characteristics of adrenocortical carcinoma(ACC)with hypercortisolism and analyze the prognostic factors.Methods Clinical data of ACC patients between January 2003 and December 2022 from Nanjing Drum Tower Hospital were collected retrospectively.Clinical and pathological characteristics were compared between ACC patients with hypercortisolism and nonfunctional ACCs.Kaplan-Meier method was used for survival analyses and Cox regression models were performed to analyze prognostic factors for ACC patients.Results In 61 cases of ACC patients,the average age was(49.33±16.32)years.After a median follow-up of 77 months(95%CI 47.49-106.51 months),median overall survival and progression-free survival were 50 months(95%CI 20.44-79.56 months)and 29 months(95%CI 22.87-35.13 months),respectively.Among 36 patients with complete endocrinologic evaluations,19(52.77%)were diagnosed with hypercortisolism.Compared with nonfunctional ACC,patients with hypercortisolism had more hypokalemia(42.11%vs 6.25%,P=0.022),capsular invasion(68.42%vs 25.00%,P=0.018),and distant metastases(73.68%vs 25.00%,P=0.007).Median overall survival and progression-free survival were significantly shorter than nonfunctional ACC(overall survival:39 months vs 67 months,P=0.009;progression-free survival:30 months vs 51 months,P=0.040)as well.Multivariate Cox regression analyses indicated that Ki67 index(HR=1.078,P=0.024)was an independent risk factor for overall survival.Hypercortisolism(HR=71.112,P=0.006),Ki67 index(HR=1.345,P=0.003),adjuvant therapy(HR=176.652,P=0.012),and operation(HR=0.020,P=0.003),were associated with disease progression.Conclusion ACC accompanied by hypercortisolism is more prone to invasion and distant metastasis,resulting in shorter survival.Hypercortisolism is an independent prognostic factor for ACC patients.