成人附睾横纹肌肉瘤合并附睾炎1例报道并文献复习  

Adult rhabdomyosarcoma of the epididymis complicated with epididymitis:a case report and literature review

作　　者：纪梓良 吴涛 陈响秋 温志强 郑清友 JI Ziliang;WU Tao;CHEN Xiangqiu;WEN Zhiqiang;ZHENG Qingyou(Department of Urology,Shenzhen Hospital,Southern Medical University,Shenzhen 518101,China)

机构地区：[1]南方医科大学深圳医院泌尿外科,广东深圳518101

出　　处：《现代泌尿外科杂志》2021年第6期489-494,共6页Journal of Modern Urology

基　　金：广东省医学科学技术研究基金项目(No.A2019268)。

摘　　要：目的提高对成人附睾横纹肌肉瘤的认识。方法报道南方医科大学深圳医院收治的1例附睾横纹肌肉瘤合并附睾炎患者的临床资料并复习总结国内外文献。结果本例患者表现为附睾肿块合并附睾炎,予抗炎治疗效果欠佳,行根治性睾丸切除术,术后病理为胚胎型横纹肌肉瘤侵犯骨髓。另总结国内外附睾横纹肌肉瘤29例,多表现为无痛性阴囊肿大,肿瘤最大径3~13 cm。19例发生远处转移,8例出现腹膜后淋巴结转移。所有病例均行根治性手术,病理主要为胚胎型(18例)。24例术后采取辅助化疗,常用VAC方案(长春新碱、阿霉素、环磷酰胺)。平均随访17.9个月,期间12例未见复发或转移,8例肿瘤无进展(带瘤生存)。9例经治疗后死亡,死亡病例中位生存期仅9个月。结论附睾横纹肌肉瘤罕见且进展快,易于早期转移,术前诊断困难,若合并附睾炎、鞘膜积液等易于混淆原发病的诊断。诊断主要依赖病理检查,早期诊断及尽早行根治性睾丸切除术辅以术后化疗是治疗的关键。Objective To improve the understanding of adult epididymal rhabdomyosarcoma.Methods The clinical data of a patient with primary epididymal rhabdomyosarcoma complicated with epididymitis were summarized and relevant literature was reviewed.Results The manifestation of this patient was an epididymal mass with epididymitis,and anti-inflammatory treatment was not effective.After radical orchiectomy,the pathology was embryonic rhabdomyosarcoma invading bone marrow.After the review of literature involving 29 cases,we found the clinical symptoms of epididymal rhabdomyosarcoma were mostly painless scrotal enlargement,with a maximum diameter of 3-13 cm.Distant metastases occurred in 19 cases,and retroperitoneal lymph node metastases occurred in 8 cases.After radical surgery,pathology showed embryonal rhabdomyosarcoma in 18 cases.Adjuvant chemotherapy of VAC regimen(vincristine,adriamycin,cyclophosphamide)was performed in 24 patients.During the average follow-up of 17.9 months,12 patients had no recurrence or metastasis,8 had no tumor progression,and 9 patients died(overall survival was 9 months).Conclusion As an extremely rare malignancy,epididymal rhabdomyosarcoma progresses quickly and may metastasize early.It's difficult to diagnose.If epididymitis or hydrocele are complicated,misdiagnosis may happen.Diagnosis depends on pathological examination.Early diagnosis,radical orchiectomy and postoperative chemotherapy are key to the treatment.

关 键 词：横纹肌肉瘤 附睾 临床特征 治疗 预后 

分 类 号：R697.2[医药卫生—泌尿科学]