颅内孤立性纤维性肿瘤/血管周细胞瘤的临床病理特征分析  

Intracranial solitary fibrous tumor/hemangiopericytoma:a clinicopathological analysis

作　　者：周兵[1] 张裕 张剑锋 曹杰[4] ZHOU Bing;ZHANG Yu;ZHANG Jian-feng;CAO Jie(Department of Pathology,Jiu Jiang No.1 People’s Hospital,Jiujiang 332000,Jiangxi,China;Center of Translational Medicine,Naval Medical University(Second Military Medical University),Shanghai 200433,China;Department of Pathology,Jiangxi Cancer Hospital,Nanchang 330006,Jiangxi,China;Center of Imaging,Jiu Jiang No.1 People’s Hospital,Jiujiang 332000,Jiangxi,China)

机构地区：[1]九江市第一人民医院病理科,九江332000 [2]海军军医大学(第二军医大学)转化医学研究中心,上海200433 [3]江西省肿瘤医院病理科,南昌330006 [4]九江市第一人民医院影像中心,九江332000

出　　处：《第二军医大学学报》2021年第11期1319-1323,共5页Academic Journal of Second Military Medical University

基　　金：江西省卫生健康委员会科技计划项目(202120056)。

摘　　要：目的探讨颅内孤立性纤维性肿瘤/血管周细胞瘤(SFT/HPC)的临床病理特征、诊断与鉴别诊断及预后。方法收集九江市第一人民医院和江西省肿瘤医院2015年6月至2020年12月确诊的7例颅内SFT/HPC患者的临床病理资料,对组织学形态、免疫组织化学染色结果进行回顾性分析。结果7例颅内SFT/HPC中男5例、女2例,年龄为23~60岁,平均年龄为46.1岁。临床表现主要为头晕、头痛、呕吐等神经受压和颅内压增高症状。影像学显示颅内占位性病变伴动脉期强化。5例WHOⅡ级SFT/HPC镜下见肿瘤由短梭形细胞构成,伴瘤组织间"鹿角样"血管及纤维组织增生;2例WHOⅢ级SFT/HPC镜下瘤细胞明显丰富且存在异型性。免疫组织化学染色显示,7例患者肿瘤细胞均弥漫表达波形蛋白、CD34和信号转导与转录激活因子6(STAT6),不同程度表达Bcl-2(3/7)、P53(3/7)、CD99(2/5)。除1例患者转院后失访外,其余6例随访5~67个月,其中1例患者于术后33个月复发,行二次手术后未见复发和转移。结论颅内SFT/HPC较为罕见且临床及影像学表现不典型,易与其他颅内肿瘤相混淆,确诊依靠其特征性的组织学形态及免疫组织化学标志物。早期诊断和完全性手术切除及放射治疗能减少SFT/HPC复发。Objective To investigate the clinicopathological features,diagnosis,differential diagnosis and prognosis of intracranial solitary fibrous tumor/hemangiopericytoma(SFT/HPC).Methods The clinicopathological data of 7 patients with intracranial SFT/HPC diagnosed in JiuJiang No.1 People’s Hospital and Jiangxi Cancer Hospital from Jun.2015 to Dec.2020 were collected,and the histological morphology and immunohistochemistry results were analyzed retrospectively.Results Of the 7 cases of intracranial SFT/HPC,5 were males and 2 were females,aged from 23 to 60 years,with a mean age of 46.1 years.The main clinical manifestations were dizziness,headache,vomiting and other symptoms of nerve compression and increased intracranial pressure.Imaging showed intracranial space occupying lesions with arterial phase enhancement.Microscopically,the tumors in 5 cases of World Health Organization(WHO)gradeⅡSFT/HPC were composed of short spindle cells with interstitial“antler like”vascular and fibrous hyperplasia;the tumor cells in 2 cases of WHOⅢSFT/HPC were significantly abundant and heterotypic.Immunohistochemical staining showed that vimentin,CD34,and signal transducer and activator of transcription 6(STAT6)were diffusely expressed in tumor cells of 7 patients,and Bcl-2(3/7),P53(3/7)and CD99(2/5)were expressed in varying degrees.Except for 1 patient transferred to other hospital and lost,the other 6 patients were followed up for 5 to 67 months.One patient recurred 33 months after the operation,and there was no recurrence or metastasis after the secondary operation.Conclusion Intracranial SFT/HPC is rare.With atypical clinical and imaging manifestations,it is easy to be confused with other intracranial tumors.The diagnosis depends on its characteristics of histological morphology and immunohistochemical markers.Early detection,complete surgical resection and radiotherapy can reduce recurrence of SFT/HPC.

关 键 词：颅内肿瘤 孤立性纤维性肿瘤 血管周细胞瘤 临床病理特征 

分 类 号：R739.41[医药卫生—肿瘤]