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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 pathological features, diagnosis and differential diagnosis of bladder primary alveolar soft tissue sarcoma. Methods The clinical data of one case of consultation were collected, routine HE staining and immunohistochemical staining were performed. The related literatures were reviewed and combined with the clinical features. Results Children with children, 12 years old. Due to painless gross hematuria, frequent urination 3 days treatment. Ultrasonography revealed a low echo mass in the left posterior wall of the bladder with a size of 4.3 cm × 3.7 cm. Transurethral resection of bladder tumor. Microscopic tumor cells were epithelial, tumor cells arranged in nests and alveoli, cytoplasm is rich, eosinophilic, interstitial sinusoidal rich, visible hemosiderin deposition. Immunohistochemistry: The tumor cells TFE3 (+), CK, CgA, Syn, vimentin, desmin, myogenin, MyoD1, S-100, HMB45 and melanA all (-). Conclusions Primary alveolar soft tissue sarcoma in the bladder is extremely rare. The tumor has unique pathomorphological features. TFE3 has high sensitivity and specificity. It should mainly be associated with urothelial carcinoma, paraganglioma, Cell tumor and metastatic Xp11.2 translocation / TFE3 gene fusion associated renal cell carcinoma and other phase identification.