CAJ | 학술논문

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.
목적 제고방광염성기섬유모세포류적진치수평.방법 방광염성기섬유모세포류환자7례.남3례,녀4례.평균년령38(10～54)세.림상주요표현위뇨통20～60 d(평균33 d)、혈뇨3～20 d(평균9 d),종류위우방광좌측벽3례,정저부4례.B초검사균제시저회성실질성단괴,대소1.4 cm×1.2 cm～4.7 cm×4.5 cm.CT검사표현위불규칙혹류원형연조직저밀도영.방광경검사제시1.5 cm×1.5 cm～5.0 cm×5.0 cm대소채화상종물,기저부교관,표면유배사조직급혈괴부착2례.방광경하취활검3례,2례보고이행세포암Ⅰ급,1례보고방광이행세포유두상증생,소조성구역정악성잠능미정,유두상종류개변.술중행빙동병리절편2례,1례보고방광육류,중저도악성;1례보고방광간협조직종류,저도악성.결과 7례균행방광부분절제술.병리진단방광염성기섬유모세포류.종류1.5 cm×1.5 cm～5.0 cm×5.0 cm대소,다위회갈색,질연,유광택,수종명현.경하가견명현점액변구화부우세포구,사형、단사형급성망상기섬유모세포산재우점액양기질중,이사형최상견,포질기이홍,병반대량장세포、기산성립세포、중성립세포급림파세포침윤.면역조직화학염색7례평활기기동단백、파형단백、AE1/AE3양성,간변성림파류격매양성6례,개결합단백양성4례,결단백양성6례,CD_(34)、CD_(117)급S-100단백균음성.6례평균수방35(1～60)개월,균존활,무복발.결론 방광염성기섬유모세포류시일충중간형병구악성잠능적종류.림상표현、영상학검사、방광경검사무특이성,병리검사급면역조화염색가학진,치료이수술절제위주.
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.