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

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。

Objective To explore the clinicopathological characteristics,immunophenotype,main points of diagnosisand differential diagnosis,treatment and prognosis of retroperitoneal solitary fibrous tumor(SFT).MethodsFour cases of retroperitoneal solitary fibrous tumor who were admitted to Wuhan Third Hospital from January 2015 to December 2018 were collected for histopathological observation and immunohistochemistry.Summarize the clinical and imaging symptoms,histological morphology,immunophenotypic 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.ResultsFour cases of retroperitoneal solitary fibrous tumor were diagnosed as"asymptomatic retroperitoneal tumors".The pathological feature was thatthe tumors were composed 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.ConclusionsThe retroperitoneal solitary fibrous tumor is an intermediate tumor,which is relatively rare in clinical practice.It is difficult to diagnose by imaging examination,lack of preoperative diagnosis basis,and easily misdiagnosed or missed diagnosis.Diagnosis and differential diagnosis must be based on pathological diagnosis and immunohistochemistry.The clinical prognosis is good,and there is a rare recurrence after complete resection,but long-term follow-up is required.