泌尿生殖系统孤立性纤维瘤的临床特征和治疗策略

Clinical characteristics and therapeutic strategy of urogenital solitary fibrous tumors

目的分析、总结并探究泌尿生殖系统孤立性纤维瘤(SFT)的诊断方法及治疗策略。方法报道1例男性泌尿生殖系统SFT病例,并总结该患者的诊治过程。回顾2008年至2018年国内外报道的总计35例患者的人口学特征、临床和病理特征、治疗及随访情况。结果35例患者中有24例(68.6%)具有相关泌尿系统症状,而11例(31.4%)在确诊时无明显不适主诉。在病理结果方面,34例(97.1%)患者的手术病理标本表现为CD34^+,此外27例(77.1%)的手术病理标本表现为Bcl-2^+。结论磁共振成像(MRI)和计算机断层扫描(CT)是检查SFTs的最有效影像学方法;手术病理标本的免疫组化是明确诊断的关键,尤其是CD34、Bcl-2、STAT6、ALDH2的特征性染色对明确诊断有重要价值;此外,分子病理学检测NAB2-STAT6融合蛋白的存在亦可提示SFTs的诊断。手术切除可能是SFTs最好的治疗方法。

Objective To analyze,summarize and explore the diagnostic methods and treatment strategies of urogenital solitary fibrous tumor(SFT).Methods The study was carried out by reporting a male patient with urogenital SFT,and by reviewing the demographic,clinical and pathological features,treatment and follow-up of 35 patients reported from 2008 to 2018.Results Of the 35 patients,24(68.6%)had urinary symptoms,while 11(31.4%)had no obvious complaints of discomfort at the time of diagnosis.In terms of pathological results,the surgical and pathological specimens of 34 patients(97.1%)were CD34^+,and 27 cases(77.1%)were Bcl-2^+.Conclusions The MRI and CT are efficient technology for detection of the SFTs.The immunohistochemical test of surgical specimens is the key to establish diagnosis,especially the characteristic staining of CD34,Bcl-2,STAT6 and ALDH2 is of great value in the diagnosis.In addition,the detection of NAB2-STAT6 fusion protein by molecular pathology also facilitate the diagnosis of SFTs.Surgical resection is the best therapy for SFTs.