ALK、TRK及ROS1在Spitz肿瘤中的表达及与临床和组织病理学特征的关系  

Expression of common kinase proteins ALK,TRK and ROS1 in Spitz tumors and their associations with clinical and histopathological characteristics of Spitz tumors

作　　者：张聪聪 邵雪宝 张莹 宋昊 王小坡 张韡 徐秀莲 姜祎群 曾学思 孙建方 臧洁 陈浩 Zhang Congcong;Shao Xuebao;Zhang Ying;Song Hao;Wang Xiaopo;Zhang Wei;Xu Xiulian;Jiang Yiqun;Zeng Xuesi;Sun Jianfang;Zang Jie;Chen Hao(Department of Pathology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)

机构地区：[1]中国医学科学院,北京协和医学院皮肤病医院病理科,南京210042

出　　处：《中华皮肤科杂志》2024年第9期807-814,共8页Chinese Journal of Dermatology

基　　金：国家重点研发计划项目(2022YFC3601800);江苏省自然科学基金(BK20211026);江苏省卫健委医学科研项目(M2021045);中国医学科学院医学与健康科技创新工程临床转化专项(2023-12M-C&T-B-110)。

摘　　要：目的研究常见激酶蛋白间变性淋巴瘤激酶(ALK)、原肌球蛋白受体激酶(TRK)及C-ROS肉瘤致癌因子-受体酪氨酸激酶(ROS1)在Spitz肿瘤中的表达及其与临床和组织病理学特征的关系。方法回顾性分析2017年1月至2023年8月在中国医学科学院皮肤病医院病理科收集的Spitz肿瘤患者的临床及病理特征并随访,对病理组织进行ALK、pan-TRK及ROS1免疫组化染色,探讨各激酶蛋白与临床和组织病理的关系。结果Spitz肿瘤57例,女36例,男21例,其中ALK阳性30例(52.6%),ROS1阳性4例(7.0%),TRK阳性仅2例(3.5%),三者均阴性21例(36.8%)。ALK阳性30例,中位年龄9.5岁,21例(70.0%)为女性,15例(50.0%)皮损位于面部,皮损表现为丘疹或结节;组织学上,29例(96.7%)肿瘤外生性生长,色素较少,肿瘤细胞以大的长梭形细胞为主,呈长条索状、丛状或束状排列。ROS1阳性4例,女3例,男1例,表现为外生性丘疹或息肉;组织学上肿瘤细胞多呈小巢状分布,细胞巢周裂隙不明显。TRK阳性2例分别为20岁及50岁男性患者,分别表现为褐色及肤色扁平丘疹;组织学上,肿瘤较表浅,基底较平,表皮内肿瘤细胞呈Paget样播散,部分细胞呈上皮样,可见小的细胞巢。染色均阴性的21例中,男9例,女12例,临床表现为斑疹、丘疹及息肉状;组织学上多数肿瘤较表浅,由上皮样细胞及梭形细胞混合组成,异型性及核分裂象少见,2例出现轻度组织结构及细胞不典型。57例随访2~83.3个月(中位随访时间为19.2个月),除1例ALK染色阳性儿童病例出现1次复发外,其余无复发或淋巴结转移。结论本组Spitz肿瘤中,激酶蛋白ALK阳性表达率最高,TRK及ROS1染色阳性病例为散发。组织病理学上,ALK染色阳性Spitz肿瘤细胞多呈长梭形条索状或丛状生长模式,TRK及ROS1染色阳性病例肿瘤细胞可能更倾向出现小的细胞巢结构。激酶蛋白染色阳性与染色阴性Spitz肿瘤病例多预后良好。Objective To investigate the expression of anaplastic lymphoma kinase(ALK),tropomyosin receptor kinase(TRK),and recombinant C-Ros oncogene 1,receptor tyrosine kinase(ROS1)in Spitz tumors,and to analyze their associations with clinical and histopathological features of Spitz tumors.Methods Clinical and histopathological characteristics,as well as follow-up data,were collected from patients with Spitz tumors at Department of Pathology,Hospital of Dermatology,Chinese Academy of Medical Sciences from January 2017 to August 2023,and retrospectively analyzed.Immunohistochemical staining for ALK,pan-TRK,and ROS1 was performed on skin tissues,and associations between the expression of these kinase proteins and clinicopathological features were analyzed.Results A total of 57 patients with Spitz tumors were collected,including 36 females and 21 males.Immunohistochemical staining showed that 30(52.6%)patients were positive for ALK,4(7.0%)were positive for ROS1,only 2(3.5%)were positive for TRK,and 21(36.8%)were negative for the three kinase proteins.Among the 30 ALK-positive patients,the median age was 9.5 years,21(70.0%)were females,and 15(50.0%)presented with lesions on the face,which mainly manifested as papules or nodules;histologically,29(96.7%)patients had hypopigmented tumors with an exophytic growth pattern,and the tumor cells were mainly large and long spindle cells arranged in long cord-like,plexiform or fascicular patterns.Among the 4 ROS1-positive patients,there were 3 females and 1 male,presenting with exophytic papules or polyps;histologically,tumor cells were mostly arranged in small nests,without obvious clefts around cell nests.Two TRK-positive patients were both males aged 20 and 50 years respectively,and presented with brown and skin-colored flat papules,respectively;histologically,the tumors were located superficially with a flat base,and tumor cells spread in a pagetoid pattern in the epidermis,with some epithelioid cells and small cell nests.Among the 21 patients negative for the 3 kinase proteins,9 we

关 键 词：痣 上皮样和梭形细胞 Spitz肿瘤 组织病理学 激酶融合基因 预后 

分 类 号：R739.5[医药卫生—肿瘤]