涎腺腺样囊性癌40例临床病理特征及预后分析

Clinicopathological features and prognosis of salivary adenoid cystic carcinoma:an analysis of 40 cases

目的探讨涎腺腺样囊性癌(salivary adenoid cystic carcinoma,SACC)的临床病理学特征及其与预后的关系。方法回顾性分析40例SACC的临床病理资料,采用免疫组化EnVision两步法检测Ki-67、CD117、CK19、p63、SMA、S-100、β-catenin、LEF-1及D2-40的表达;FISH法检测MYB-NFIB融合基因。采用Kaplan-Meier法进行生存分析,单因素和多因素Cox生存分析预后因子。结果40例SACC中36例(90%)SACC镜下可见肿瘤周围收缩裂隙(peritumoral retraction cleft,PRC),D2-40显示PRC非脉管内瘤栓。14例SACC中8例(57.1%)MYB-NFIB融合基因阳性。39例SACC随访3~114个月,术后中位总生存时间为78个月,5、8、10年生存率为77.8%、26.4%及0;术后中位无瘤生存时间为51个月,2、5及8年无瘤生存率分别为78.5%、40.8%及0。预后分析显示,Ki-67增殖指数>15%(HR=3.943,P=0.037)、PRC(HR=2.469,P=0.040)与未根治性切除(HR=2.526,P=0.043)为独立影响变量。结论PRC见于90%的SACC,可作为病理诊断参数。Ki-67增殖指数>15%、PRC与未根治性切除为SACC的独立危险因素。

Purpose To summarize the clinicopathological characteristics of salivary adenoid cystic carcinoma(SACC)and its association with prognosis.Methods A retrospective analysis of the clinicopathological data of 40 SACC cases was performed.Ki-67,CD117,CK19,p63,SMA,S-100,β-catenin,LEF-1 and D2-40 were detected by immunohistochemistry(IHC),and MYB-NFIB fusion gene was detected by FISH.Kaplan-Meier curve was used for survival analysis,and univariate and multivariate Cox regression were used to analyze prognostic factors.Results Thirty-six cases(90%)showed peritumoral retraction cleft(PRC)under microscope,and D2-40 showed that PRC was not an intravascular tumor thrombus.Eight of the 14 SACCs(57.1%)were positive for the MYB-NFIB fusion gene.Thirty-nine SACC cases were followed up for 3 to 114 months.The median survival time after surgery was 78 months.The 5-year,8-year,and 10-year survival rates were 77.8%,26.4%,and 0,respectively.The median time of postoperative tumor-free survival was 51 months,and the 2-year,5-year,and 8-year tumor-free survival rates were 78.5%,40.8%,and 0,respectively.Prognostic analysis showed that Ki-67>15%(HR=3.943,P=0.037),PRC(HR=2.469,P=0.040)and uncured resection(HR=2.526,P=0.043)were independent variables.Conclusion PRC is seen in 90%of SACC cases and can be used as a pathological diagnosis parameter.Ki-67 index>15%,PRC and uncured resection are independent risk factors for SACC.