肾脏球旁细胞瘤6例临床病理特征分析

Clinicopathological features of renal juxtaglomerular cell tumor:analyses of six cases

目的探讨肾脏球旁细胞瘤(juxtaglomerular cell tumor,JGCT)的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析6例JGCT的临床病理学资料,对切除标本进行常规病理组织学、免疫组化、特殊染色及电镜检查,并复习相关文献。结果6例患者中4例女性,2例男性,年龄32~53岁,2例发生于左侧肾脏,4例发生于右侧肾脏;患者均有持续性、顽固性高血压病史,影像学示肾脏占位。眼观:肿瘤边界清楚,切面呈黄褐色,有完整的纤维性包膜。镜检:瘤细胞呈实性片状分布,中间可见残存肾小管。部分形成血管外皮瘤样结构。间质中血管丰富,血管壁厚、玻璃样变性。同时可见肥大细胞和淋巴细胞浸润,瘤细胞之间界限较清,有颗粒状嗜酸性胞质。部分细胞核周有空晕,细胞核圆形,部分可见核仁,核分裂少见。免疫表型:瘤细胞表达CD34、vimentin和Renin,部分弱表达SMA;Bowie、PAS和甲苯胺蓝染色均阳性,电镜示瘤细胞内见成簇或散在的大小不一的颗粒状结构,有的呈菱形或扁菱形结晶样。结论JGCT临床较罕见,患者多伴高血压病史,属于具有特殊功能的神经内分泌肿瘤。确诊需结合病理学形态及免疫表型;大部分病例表现为良性生物学行为,预后较好。

Purpose To investigate the clinicopathological characteristics,immunophenotype and differential diagnosis of renal juxtaglomerular cell tumor(JGCT).Methods The clinicopathological data of 6 cases of renal paracytoma were analyzed retrospectively.The specimens were examined by routine histopathology,immunohistochemistry,special staining and electron microscopy.Results All 6 cases were adults,aged 32-53 years,4 cases were female,2 cases were male,2 cases occurred in the left kidney,and 4 cases occurred in the right kidney.The patients had a clinical history of persistent refractory hypertension,imaging findings of renal occupation.The boundary of the tumor was clearly observed,the cut surface was yellowish brown,and there were many intact fibrous capsule.Microscopically,the tumor cells showed a solid patchy distribution with residual renal tubules in the middle.Part of the hemangiomatoid structure was formed.The interstitium was rich in blood vessels with thick vascular wall and hyaline degeneration.Infiltration of mast cells and lymphocytes was also seen.The cell boundaries were relatively clear,with granular eosinophilic cytoplasm.Part of the nucleus was perianatomic halo,the nucleus was round,part of the nucleolus was visible,and nuclear fission was rare.Immunohistochemical markers showed that tumor cells expressed CD34,vimentin and renin,and partial SMA was weakly positive.Bowie staining,PAS staining and toluidine blue staining were positive,and electron microscopy showed that there were many clusters or scattered secretory particles in tumor cells,some of which showed diamond or flat diamond crystals.Conclusion JGCT is rare in clinic,and most patients have a history of hypertension.It is a neuroendocrine tumor with special function.The diagnosis should be combined with histological morphology and immunohistochemistry.Most cases show benign biological behavior and have a good prognosis.