文章摘要
金冬梅,夏琛.腹膜后孤立性纤维性肿瘤 4例临床病理分析[J].安徽医药,2020,24(7):1388-1390.
腹膜后孤立性纤维性肿瘤 4例临床病理分析
Clinicopathological analysis of 4 cases of retroperitoneal solitary fibrous tumors
  
DOI:10.3969/j.issn.1009?6469.2020.07.030
中文关键词: 腹膜后肿瘤  病理状态,体征和症状  S100蛋白质类  抗原, CD34  孤立性纤维性肿瘤  诊断  预后
英文关键词: Retroperitoneal neoplasms  Pathological conditions,signs and symptoms  S100 Proteins  Antigens,CD34  Soli? taryfibrous tumor  Diagnosis  Prognosis
基金项目:
作者单位
金冬梅 武汉市第三医院病理科湖北武汉 430060 
夏琛 武汉市第三医院病理科湖北武汉 430060 
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中文摘要:
      目的探讨腹膜后孤立性纤维性肿瘤( Solitary Fibrous Tumor,SFT)的临床病理特征、免疫表型、诊断与鉴别诊断要点、治疗及预后。方法收集 2015年 1月至 2018年 12月就诊于武汉市第三医院的 4例腹膜后孤立性纤维性肿瘤进行组织病理学观察及免疫组织化学检测,总结其临床及影像学表现、组织学形态、免疫表型特征和临床预后。免疫组织化学采用 EnVision法检测内皮细胞标记物( CD34)、 B淋巴细胞瘤 ?2基因( Bcl?2)、 CD99分子重组蛋白( CD99)、信号转导和转录活化因子 6(STAT6)及其他相关标志物。并结合相关文献进行讨论分析。结果 4例腹膜后孤立性纤维性肿瘤均是因“无症状腹膜后肿物”就诊,病理特征为肿瘤由梭形、短梭形细胞构成,排列无序并与胶原纤维混杂,肿瘤中血管丰富,部分形成血管外皮瘤样区域,免疫组化 STAT6(+)、 CD34(+)、 Bcl?2(+)、 CD99(+)、人 S100蛋白( S?100)(-)、酪氨酸激酶生长因子受体蛋白( CD117)(-)、 Discovered on GIST?1(Dog?1)(-)、细胞角蛋白( CK)(-)、结蛋白( Desmin)(-)、黑色素瘤抗体( HMB45)(-)、上皮膜抗原( EMA)(-)。 1例失访,余均未复发。结论腹膜后孤立性纤维性肿瘤为中间型肿瘤,临床上比较少见,难以通过影像学检查确诊,缺乏术前诊断依据,极易误诊、漏诊,须依据病理诊断,免疫组化进行诊断与鉴别诊断。该病临床上预后较好,完整切除后罕见复发,但需长期随访。
英文摘要:
      Objective To explore the clinicopathological characteristics,immunophenotype,main points of diagnosisand differential diagnosis,treatment and prognosis of retroperitoneal solitary fibrous tumor(SFT).Methods Four cases of retroperitoneal solitaryfibrous tumor who were admitted to Wuhan Third Hospital from January 2015 to December 2018 were collected for histopathologi?cal observation and immunohistochemistry.Summarize the clinical and imaging symptoms,histological morphology,immunophenotyp? ic characteristics and clinical prognosis.For Immunohistochemistry,EnVision were used to detect CD34,Bcl?2,CD99,STAT6,and other related markers.And combined with relevant literature for discussion and analysis.Results Four cases of retroperitoneal soli?tary fibrous tumor were diagnosed as "asymptomatic retroperitoneal tumors".The pathological feature was thatthe tumors were com?posed ofspindle cells and short spindle cells,and arranged disorderly and mixed with collagen fibers.The tumor are rich in blood vessels,and partly formed an hemangiopericytoma?like area.The immunohistochemical results:The cells express STAT6,CD34,Bcl? 2 and CD99,andcannot expressS?100、CD117、Dog?1、CK、Desmin、HMB45、EMA.One case was lost to follow?up and none of them relapsed.Conclusions The retroperitoneal solitary fibrous tumor is an intermediate tumor,which is relatively rare in clinical prac? tice.It is difficult to diagnose by imaging examination,lack of preoperative diagnosis basis,and easily misdiagnosed or missed diag?nosis.Diagnosis and differential diagnosis must be based on pathological diagnosis and immunohistochemistry.The clinical prognosisis good,and there is a rare recurrence after complete resection,but long?term follow?up is required.
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