22例肾上腺皮质癌病理特征及临床价值

目的:总结分析肾上腺皮质癌(adrenal cortical carcinoma,ACC)临床、组织病理及免疫组织化学染色特征,并评估病理特点对患者预后的预测价值。方法:回顾性分析2010年1月至2018年12月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。结果:22例ACC患者病理组织标本表现为瘤体形状不规则,内部可见出血坏死区域;其中包膜侵犯者14例,存在点片状钙化者10例。所有病例Weiss评分≥3。免疫组织化学检查结果显示Ki-67指数5%~70%,Vim阳性者94.74%(18/19),Syn阳性者94.12%(16/17),CR阳性者90.91%(10/11),CD34阳性者80.00%(8/10),NSE阳性者71.43%(10/14),Melan A阳性者60.00%(9/15),Inhi b in阳性者53.33%(8/15),CK阳性者33.33%(6/18),Cg A阳性者6.67%(1/15)。发生远处转移的患者Ki-67指数显著高于无转移者(28.83±8.95)%vs(10.91±2.41)%,P=0.025]。利用ROC曲线分析Ki-67指数对预测肿瘤远处转移的价值,晚期患者的ROC曲线下面积为0.86(P=0.03)。当Ki-67指数>6.5%时,其敏感性为100%,特异性为50%;当Ki-67指数>12.5%时,其敏感性为80%,特异性为80%。利用K-M生存曲线评估预后情况,结果示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(中位生存期30个月vs 8个月,P=0.006)。对于接受NCCN指南推荐治疗方案的患者,Ki-67指数<20%的患者中位生存期优于Ki-67指数≥20%的患者(50个月vs6个月,P=0.011)。结论:病理组织检查是ACC诊断的金标准,发生远处转移的患者Ki-67指数显著高于无转移者,Ki-67指数可用于预测晚期ACC预后。

Pathological features and clinical value of 22 cases of adrenal cortical carcinoma

Objective:To investigate the clinical features,histopathological and immunohistochemical staining characteristics of adrenal cortical carcinoma(ACC),and to evaluate the predictive value of prognosis based on pathological features.Methods:The clinicopathological features of 22 ACC patients admitted to the First Affiliated hospital of Xi’an Jiaotong University from January 2010 to December 2018 were retrospectively analyzed.Results:All tumors were irregularly shaped,vwith necrosis 14 cases had capsular invasion and 10 had calcification.The Weiss scores of all cases were≥3.Immunohistochemical results showed Ki-67 index varied from 5%to 70%.Eighteen cases were Vimentin(Vim)positive,16 were synaptophysin(Syn)positive and 9 were MelanA positive.Inhibin,CR,NSE and CD34 were positive in 8,10,10 and 8 cases respectively.Six cases were CK positive and 1 was CgA positive.The Ki-67 index in cases with distant metastasis were significantly higher than that in cases without distant metastasis(28.83±8.95)%vs(10.91±2.41)%,P=0.025].ROC curve was used to analyze the power of Ki-67 index in predicting distant metastasis.The area under the ROC curve of end-stage patients was 0.86(P=0.03).A cut-off value of 6.5%resulted in sensitivity of 100%and specificity of 50%.The sensitivity and specificity were both 80%when using a cut-off value of 12.5%.Kaplan-Meier survival curve was used to evaluate prognosis.Patients undergoing adrenal tumor resection had a significantly better long-term outcome than those without resection(Median survival 30 months vs 8 months,P=0.006).For patients receiving standardized treatment recommended by the NCCN guideline,patients with Ki-67 index<20%had a higher median survival than those with Ki-67 index≥20%(50 months vs 6 months,P=0.011).Conclusion:Pathological examination is the gold standard for ACC diagnosis.Ki-67 index is significantly higher in patients with distant metastasis and can be used to predict the prognosis of late stage ACC.