目的:探讨临床特点,形态学和免疫组织化学特征,诊断,鉴别诊断,肾近肾小球细胞肿瘤(JGCT)的组织发生和预后。
方法:光镜观察;CK8,E-cadherin/CK7,CD10,Vim,肌动蛋白,CD34,S100,HMB45,CD31,Chr,Syn和CD117,EM;对所有4例经手术治疗的JGCT患者进行了随访。
结果:所有4例JGCT均在临床未控制的重度高血压的年轻成人中观察到。严重的,肿瘤被包裹,体积较小.微观上,肿瘤细胞主要生长在片状,但是也可以看到乳头状和洋葱状的图案。基质包含突出的脉管系统,由许多聚集在厚壁血管周围的薄壁血管组成。肿瘤细胞相当小,多边形,微嗜酸性细胞浆和细胞边界不明确。细胞核大小均匀,但可以看到核异型和有丝分裂。许多肥大细胞散布在肿瘤细胞中,4例中有3例远端小管标志物E-cadherin/CK7阳性表达。肿瘤细胞阳性表达Vim,肌动蛋白,Calponin,CD34所有病例均表现出明显的菱形晶体的超微结构特征。所有4例JGCT患者在随访期间(平均37个月)均无复发或转移,但高血压持续存在。
结论:JGCT,起源于近球细胞,有一个独特的良性实体,它通常在患有严重高血压的年轻人中发现。它具有独特的形态学和超微结构特征以及对Vim的阳性免疫反应性,肌动蛋白,calponin和CD34。
OBJECTIVE: To investigate the clinical characteristics, morphologic and immunohistochemical features, diagnosis, differential diagnosis, histogenesis and prognosis of renal juxtaglomerular cell tumor (JGCT).
METHODS: Light microscopic observation; immunohistochemical assay of CK8, E-cadherin/CK7, CD10, Vim, Actin, CD34, S100, HMB45, CD31, Chr, Syn and CD117, EM; and follow-up were done on all 4 surgically treated JGCT patients.
RESULTS: All 4 JGCT were observed in young adult with clinically uncontrolled severe hypertension. Grossly, the tumor was encapsulated and small in size. Microscopically, the tumor cells grew in sheets predominantly, but papillary and onion-like pattern could also be seen. The stroma contained prominent vasculature that consisted of numerous thin-wall vessels clustering around thick-walled vessels. Tumor cells were rather small, polygonal, with slightly eosinophilic cytoplasm and ill-defined cell border. Nuclei were uniform in size but nuclear atypia and mitosis could be seen. Numerous mast cells were scattered among the tumor cells, and tubules were identified in 3 of 4 cases with positive expression of distal tubule marker of E-cadherin/CK7. Tumor cells positively expressed Vim, Actin, calponin, and CD34. All cases presented ultrastructural features of distinct rhomboid-shaped crystal. There was no recurrence or metastasis but hypertension persisted in three during follow-up (mean 37 months) for all 4 JGCT patients.
CONCLUSIONS: JGCT, originating from the juxtaglomerular cell, has a distinct benign entity, and it is typically found in young adults with severe hypertension. It has a unique morphology and ultrastructure features and positive immunoreactivity to Vim, Actin, calponin and CD34.