膀胱平滑肌肉瘤3例报告并文献复习

Leiomyosarcoma of the urinary bladder:3 cases report and review of the literature

目的探讨膀胱平滑肌肉瘤的临床病理和诊治方法。方法患者3例,肉眼血尿,膀胱镜检见肿瘤分别发生于膀胱左侧壁、左前壁和右后壁,直径2.0～3.5cm,以菜花样和乳头样为主,基底较宽,瘤体表面光滑。结果 3例均行全膀胱切除术,病理表现为上皮下间质富于黏液,黏液中可见散在或束状分布的细长梭形瘤细胞,其胞质淡染,核呈长梭形,有轻度异型,核分裂象易见。免疫组化SMA(+)3例、MSA(+)3例、Des(+)2例、S-100(-)2例、CD34(-)3例,均诊断为膀胱低级别平滑肌肉瘤。随访6个月～8年,1例术后2.5年死亡,2例存活。结论膀胱平滑肌肉瘤临床罕见,确诊依赖病理及免疫组化检查,应与平滑肌瘤、横纹肌肉瘤、癌肉瘤、炎性肌纤维母细胞瘤等鉴别,治疗以手术为主,需根据肿瘤分级、分期决定手术方案及术后是否需行辅助治疗。

Objective To review the clinical features and treatment of leiomyosarcoma in urinary bladder.Methods 3 cases with gross hematuria were reported,the lesions were seen on left lateral,left anterior wall or right posterior wall by cystoscope,2.0 to 3.5 cm diameter,cauliflower-like or papillary with broad basilar part and smooth surface.Results After cystectomy and urinary diversion,the pathological results showed abundant mucus in the mesenchyma,composed of spindle cells arranged in a vaguely fascicular pattern with light stained kytoplasm,which nucleus were spindle shaped with light atypia and mitotic rate was brisk,immunohistochemical results showed SMA（＋）（3 cases）,MSA（＋）（3 cases）,Des（＋）（2 cases）,S-100（-）（2 cases）,CD34（-）（3 cases）,which confirmed a diagnosis of leiomyosarcoma of the urinary bladder.After follow-up for 6 months to 8 years,one died for 2.5 years after operation and two live yet without recurrence or metastasis.Conclusions The leiomyosarcoma of bladder is rare,diagnosed mainly on pathological and immunohistochemical diagnosis and should be differentiated from the leiomyoma,rhabdomyosarcoma,carcinosarcoma and inflammatory myofibroblastic tumor.The main treatment is cystectomy and whether adjuvant therapy or not shoud accord with tumor grade and stage.