泌尿生殖系统孤立性纤维瘤的临床特征和治疗策略

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。

Clinical characteristics and therapeutic strategy of urogenital solitary fibrous tumors

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.