四例肾球旁细胞瘤的临床病理学观察

Clinicopathological study of 4 renal juxtaglomerular cell tumors

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 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 hypertention persisted in three during follow-up (mean 37months) for all 4 JGCT patients. Conclusion 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.