膀胱炎性肌纤维母细胞瘤的诊断与治疗

目的 提高膀胱炎性肌纤维母细胞瘤的诊治水平.方法 膀胱炎性肌纤维母细胞瘤患者7例.男3例,女4例.平均年龄38(10～54)岁.临床主要表现为尿痛20～60 d(平均33 d)、血尿3～20 d(平均9 d),肿瘤位于膀胱左侧壁3例,顶底部4例.B超检查均提示低回声实质性团块,大小1.4 cm×1.2 cm～4.7 cm×4.5 cm.CT检查表现为不规则或类圆形软组织低密度影.膀胱镜检查提示1.5 cm×1.5 cm～5.0 cm×5.0 cm大小菜花状肿物,基底部较宽,表面有坏死组织及血块附着2例.膀胱镜下取活检3例,2例报告移行细胞癌Ⅰ级,1例报告膀胱移行细胞乳头状增生,小灶性区域呈恶性潜能未定,乳头状肿瘤改变.术中行冰冻病理切片2例,1例报告膀胱肉瘤,中低度恶性;1例报告膀胱间叶组织肿瘤,低度恶性.结果 7例均行膀胱部分切除术.病理诊断膀胱炎性肌纤维母细胞瘤.肿瘤1.5 cm×1.5 cm～5.0 cm×5.0 cm大小,多为灰褐色,质软,有光泽,水肿明显.镜下可见明显黏液变区和富于细胞区,梭形、短梭形及星芒状肌纤维母细胞散在于黏液样基质中,以梭形最常见,胞质嗜伊红,并伴大量浆细胞、嗜酸性粒细胞、中性粒细胞及淋巴细胞浸润.免疫组织化学染色7例平滑肌肌动蛋白、波形蛋白、AE1/AE3阳性,间变性淋巴瘤激酶阳性6例,钙结合蛋白阳性4例,结蛋白阳性6例,CD_(34)、CD_(117)及S-100蛋白均阴性.6例平均随访35(1～60)个月,均存活,无复发.结论 膀胱炎性肌纤维母细胞瘤是一种中间型并具恶性潜能的肿瘤.临床表现、影像学检查、膀胱镜检查无特异性,病理检查及免疫组化染色可确诊,治疗以手术切除为主.

Inflammatory myofibroblastic tumor of the bladder

Objective To review the pathological and clinical features of inflammatory myofibroblastic tumor(IMT)of the bladder,and to discuss its diagnosis,treatment and prognosis.Methods The clinicaldata of 7 patients with IMT of the bladder were reviewed.There were 3 males and 4 females aged 10 to 54 years(mean 38).Lesions were 1.5 cm to 5.0 cm.The majority of the patients presented with hematuria.The masses of bladders were discovered by imaging studies and cystoscopys.Three cystoscopic biopsies and frozen section analysis of 2 specimens were performed,but no IMT was confirmed.Results All bladder IMTs were managed by partial cystectomy.By post-operative pathological examination,IMT of the bladder were diagnosed.By immunohistochemistry,lesions at least fotally expressed smooth muscle actin(7/7),AE1/AE3(7/7),vimentin(7/7).anaplastic lymphoma kinase(6/7),and most lacked S100(0/7),CD_(34)(017),CD_(117)(0/7).Follow-ups were available in 6 cases.There was no patient with recurrence during the follow up from 1 month to 60 months.Conclusions The IMT of the bladder is rare.The diagnosis mainly depends on pathological diagnosis.Partial cystectomy or transurethral resection could be the treatment choice.