椎管内孤立性纤维性肿瘤的临床病理学分析  

Clinicopathological features of spinal solitary fibrous tumor

作　　者：葛海静 姚晶晶[1] 李莉[1] 李博文[1] 葛翠 刘浩[1] 李勇[1] 尹洪芳[1] Ge Hajing;Yao Jingjing;Li Li;Li Bowen;Ge Cui;Liu Hao;Li Yong;Yin Hongfang(Department of Pathology,Beijing Tsinghua Changgung Hospital,School of Clinical Medicine,Tsinghua University,Beijing 102218,China)

机构地区：[1]清华大学附属北京清华长庚医院病理科清华大学临床医学院,北京102218

出　　处：《中华病理学杂志》2022年第9期875-880,共6页Chinese Journal of Pathology

摘　　要：目的探讨椎管内孤立性纤维性肿瘤(SFT)的临床病理学特点、分子遗传学及免疫组织化学特征、诊断、鉴别诊断及临床预后等。方法收集清华大学附属北京清华长庚医院2015年1月至2021年12月存档的椎管内SFT手术标本共12例, 进行回顾性研究和重新分级, 对临床影像资料、组织形态学、免疫组织化学和分子遗传学进行分析, 并进行随访及相关文献复习。结果 12例患者中男性5例, 女性7例;年龄31~73岁, 中位年龄50.5岁。12例均为原发性, 其中4例为首发病例, 8例为复发病例, WHO 1级8例、WHO 2级3例, WHO 3级1例。显微镜下表现为梭形细胞肿瘤, 间质富含大量薄壁血管, 根据肿瘤级别不同呈现不同的细胞形态及坏死等组织学特征。免疫表型上, 12例(12/12)表达波形蛋白及STAT6, STAT6均为细胞核弥漫强阳性;11例(11/12)表达CD34和bcl-2;7例(7/12)表达CD99。二代测序检测提示12例(12/12)均存在NAB2-STAT6基因融合。12例随访6~80个月, 4例首发病例术后均无复发无转移, 8例复发病例中2例再次复发, 2例死亡。结论椎管内SFT罕见, 具有较高的复发倾向, 诊断过程中需要多方面考虑, STAT6是诊断该肿瘤较为特异的标志物。完整的手术切除为首选治疗方案, 建议术后放疗以减少肿瘤复发。Objective To investigate the clinicopathological,molecular genetic,immunohistochemical and prognostic features of spinal solitary fibrous tumor(SFT).Methods The clinical data of 12 cases of spinal SFT in Beijing Tsinghua Changgung Hospital,Affiliated to Tsinghua University,diagnosed from January 2015 to December 2021 were collected and reclassified.The clinical data,histopathology,immunohistochemistry and molecular genetics were analyzed.Follow-up and related literature reviews were conducted.Results Among the 12 patients,there were 5 males and 7 females;the age ranged from 31 to 73 years,with a median age of 50.5 years.All 12 cases were primary tumors,including 4 cases diagnosed at the first time and 8 recurrent cases.Among the 12 cases,8 were WHO grade 1,3 were WHO grade 2,and 1 was WHO grade 3.Microscopically,the spinal SFT appeared as a spindle cell tumor,the stroma was rich in many thin-walled blood vessels with various histological features such as cell morphology and necrosis according to the different tumor grade.All(12/12)of the cases expressed vimentin and STAT6(diffuse and strong nuclear stain),11 cases(11/12)expressed both CD34 and bcl-2,and 7 cases(7/12)expressed CD99.Next-generation sequencing showed that 12(12/12)of the patients had NAB2-STAT6 gene fusion.The 12 patients were followed up for 6 to 80 months.There were no recurrences or metastases in the 4 first cases after operation.Among the 8 recurrent cases,2 of the patients relapsed and 2 died.Conclusions Spinal SFT is rare and has a high recurrence tendency.Many aspects need to be considered in the diagnosis process.STAT6 is a relatively specific marker for the diagnosis of this tumor.Complete surgical resection is the preferred treatment while postoperative radiotherapy is recommended to reduce tumor recurrence.

关 键 词：椎管 孤立性纤维瘤 STAT6转录因子 免疫组织化学 序列分析 

分 类 号：R739.42[医药卫生—肿瘤]