膀胱原发性腺泡状软组织肉瘤临床病理观察  

Primary alveolar soft part sarcoma in urinary bladder: a clinicolpathological study

作　　者：梁国栋[1,2] 王殿军 王昀[1] 郭爱桃[1] 罗明惠[2] 

机构地区：[1]解放军总医院病理科,北京100853 [2]呼伦贝尔市人民医院病理科

出　　处：《诊断病理学杂志》2014年第2期107-109,共3页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨膀胱原发性腺泡状软组织肉瘤的病理学形态特征、诊断与鉴别诊断。方法收集1例会诊病例的临床资料,进行常规HE染色和免疫组化染色,复习相关文献并结合临床特征综合分析。结果患儿女性,12岁。因无痛性肉眼血尿、尿频3天就诊。超声检查示膀胱左后壁低回声团,大小4.3 cm×3.7 cm。经尿道行膀胱内肿瘤电切术。镜下肿瘤细胞为上皮样,瘤细胞排列呈巢状及腺泡状,胞质丰富,嗜酸性,间质富含窦状血管,可见含铁血黄素沉积。免疫组化:肿瘤细胞TFE3(+),CK、CgA、Syn、vimentin、desmin、myogenin、MyoD1、S-100、HMB45和melanA均(-)。结论原发于膀胱的腺泡状软组织肉瘤极其罕见,该肿瘤具有独特的病理形态学特征,TFE3对其有很高的敏感性和特异性;主要应与尿路上皮癌、副节瘤、颗粒细胞瘤以及转移性Xp11.2易位/TFE3基因融合相关性肾癌等相鉴别。Objective To investigate the morphological characteristics,pathologic diagnosis and differential diagnoses of alveolar soft-part sarcoma （ASPS） that primarily occurred in the urinary bladder.Methods The clinicopathological features of a consultation case was reviewed.Routine hematoxylin-eosin and immunohistochemical staining were performed and the literatures were reviewed.Results A 12-year-old girl with a tumor in the urinary bladder was consulted.Gross hematuria and frequent urination were the main presenting symptoms.Ultrasound examination revealed a hypoechoic lesion in the left posterior of the bladder.The transurethral resection of bladder tumor was performed.Histologically,the tumor was composed of large,round,and polygonal neoplastic cells with abundant vacuolated and granular eosinophilic cytoplasm.The tumor grew in a nest-like or alveolar pattern separated by delicate fibrovascular septa.Immunohistochemically,only TFE3 was positive in the tumor cells.CK,CgA,Syn,vimentin,desmin,myogenin,MyoD1,S-100,HMB45 and melan-A were negative.Conclusion ASPS of the urinary bladder is rare,and TFE3 is a sensitive and specific marker to ASPS.When it occurs in the urinary bladder,differential diagnosis should be made among urothelial carcinoma,paraganglioma,granular cell tumor and metastatic renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.

关 键 词：腺泡状软组织肉瘤 膀胱 免疫组化 TFE3 鉴别诊断 

分 类 号：R737.14[医药卫生—肿瘤]