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目的观察肾球旁细胞瘤(JGCT)的临床特征、形态特点、免疫表型,明确其诊断、鉴别诊断、组织发生及预后。方法对4例组织标本采用光镜观察,PAS、Sweet嗜银组化染色,免疫组化检测CK8、E cadherin/CK7、CD10、Vim、Actin、desmin、calponin、CD34、HMB45、CD31、S100、Chr、Syn及CD117。电镜观察超微结构。对患者进行随访。结果4例JGCT患者临床表现为青年人难以控制的严重高血压。肿瘤包膜完整、体积较小。镜下肿瘤细胞以片状排列为主,当有肾小管结构时可见乳头、葱皮样排列;间质含丰富的薄壁血管及少量的厚壁血管;瘤细胞偏小,多角或短梭形,轻度嗜酸,胞膜不清,可见核异型及核分裂相;肿瘤内见多量的肥大细胞,3例见小管结构,并表达远端小管标志物E cadherin/CK7。肿瘤细胞Vim、Actin、CD34均为(+);4例标本电镜下均见特征性的菱形结晶。患者均无复发和转移。结论JGCT青年人多见,有严重的高血压。病理为独特的形态及超微结构,免疫表型Vim、Actin、calponin、CD34阳性,发生于肾球旁细胞,是独立类型的肾良性间叶肿瘤。
Objective To observe the clinical features, morphological features and immunophenotype of renal allograft tumor (JGCT), and to confirm its diagnosis, differential diagnosis, histogenesis and prognosis. Methods Four cases of tissue samples were examined by light microscopy, PAS, Sweet silver staining and immunohistochemical staining for CK8, E cadherin / CK7, CD10, Vim, Actin, desmin, calponin, CD34, HMB45, CD31, , Syn and CD117. Electron microscopy ultrastructure. Patients were followed up. Results The clinical manifestations of 4 patients with JGCT were severe hypertension that was difficult to control in young people. Tumor capsule integrity, smaller size. Microscopically, the tumor cells were mainly arranged in the form of lamella. When the structure of the renal tubules was seen, the papillae and onion skin were arranged. The stroma was rich in thin-walled vessels and a few thick-walled vessels. The tumor cells were small, polygonal or short fusiform , Slight eosinophilic, cell membrane unclear, visible nuclear atypia and mitotic phase; see a large number of mast cells in the tumor, 3 cases of tubular structure and expression of distal tubular marker E cadherin / CK7. The tumor cells Vim, Actin, CD34 were (+); 4 cases of specimens were observed under the characteristic rhombohedral crystal. No recurrence and metastasis of patients. Conclusion JGCT young people more common, severe hypertension. Pathology of the unique morphology and ultrastructure, immunophenotype Vim, Actin, calponin, CD34 positive, occurred in glomerular cells, is an independent type of benign mesenchymal neoplasms.