肺原发性腺样囊性癌的临床病理特征及PD-1、PD-L1、EGFR、ALK基因检测的临床意义  

Pulmonary Adenoid Cystic Carcinoma ( PACC ): Clinicopathological Features and Clinical Significance of PD-1 > PD-L1、EGFR、ALK Gene Dection

作　　者：林海峰 曲杨[1] 张海青[1] 周立娟[1] 苏丹[1] 刘子臣[1] Lin Haifeng;Qu Yang;Zhang Haiqing;Zhou Lijuan;Su Dan;Liu Ziehen(Department of Pathology,Beijing Chest Hospital,Capital Medical University,Beijing 101149,China)

机构地区：[1]首都医科大学附属北京胸科医院病理科,101149

出　　处：《结核病与胸部肿瘤》2019年第2期112-116,共5页Tuberculosis and Thoracic Tumor

摘　　要：目的探讨肺原发性腺样囊性癌(PACC)的临床病理特征及PACC中程序性死亡受体1(PD-1)及其配体1(PD-L1)、表皮生长因子受体(EGFR)、间变性淋巴瘤激酶(ALK)基因表达的临床意义。方法回顾性分析36例PACC的临床病理特征,免疫组化采用EnVision法检测PD-1及PD-L1表达情况,ARMS方法检测EGFR基因突变,VENTANAALK方法检测ALK的蛋白表达情况。结果本组36例PACC患者,男性20例,女性16例,平均发病年龄57岁。其中腺管型15例、筛状型9例、实体型12例。所有PACC患者的PD-1均呈(-),1例PD-L1呈(+)。1例EGFR基因突变(19del),靶向药物(特罗凯)治疗有效。ALK蛋白表达均为(-)。在27例手术根治标本中,晚期分期(Ⅲ-Ⅳ)、残端阳性、神经侵犯、实体/筛状型及淋巴结转移与PACC预后差有明显相关性(P<0.05)。结论PACC是一类低度恶性肿瘤,但因发病部位与主支气管密切相关,手术困难,易出现残端阳性,导致多次复发与转移。EGFR突变率低,PD-L1蛋白阳性率也很低,但此类患者在靶向治疗和生物治疗中可能获益,因此常规肺癌基因检测和PD-L1蛋白检测临床仍需重视。Objective To investigate the clinicopathological features of pulmonary adenoid cystic carcinoma ( PACC) and the clinical significance of programmed death 1 ( PD-1) and its ligand 1 ( PD-1 ligand),epidermal growth factor receptor( EGFR) and anaplastic lymphoma kinase ( ALK) gene expression in PACC.Methods Retrospective analysis of the clinical and pathological features were carried out in 36 patients with primary PACC.The expression of PD-1 and PD-L1 was detected by immunohistochemical EnVision.The ARMS was used to detect EGFR gene mutation,and the VENTANA ALK was used to detect ALK protein expression.Results Thirty-six patients were enrolled ( 20 male,16 female).The average age was 57 years.Our cohort included 15 glandular tumors,9 cribriform tumors and 12 solid tumors.PD-1 was negative in all patients,and only 1 case was positive in PD-L1 (+,1%).In this study,only one case of EGFR gene mutation ( 19del) was found,and the targeted drug ( Tarceva) was effective.No expression of ALK protein was found.In 27 cases of surgical radical specimens,advanced stage (Ⅲ-Ⅳ),margin positive,neurological invasion,solid /cribriform subtype and lymph node metastasis were significantly associated with poor prognosis ( P < 0.05).Conclusion Primary adenoid cystic carcinoma of the lung is a low-grade malignant tumor.Because its location is closely related to the trachea,the operation is difficult,and the surgical margin is always positive,leading to frequent recurrence and metastasis.Patients with PACC can get benefit from targeted therapy and immunotherapy,although both positive ratio of EGFR mutation and PD-L1 protein expression are low.Therefore,attention should be paid to routine lung cancer gene detection and PD-L1 protein detection.

关 键 词：肺肿瘤 腺样囊性癌 PD-1 PD-L1 EGFR ALK 

分 类 号：R735.2[医药卫生—肿瘤]