肾球旁细胞瘤6例临床分析及文献复习

目的探讨肾球旁细胞瘤的临床特点,提高对该病的诊断和治疗水平。方法回顾性分析6例肾球旁细胞瘤患者临床资料并复习相关文献。男5例,女1例,初诊年龄26.2±5.7（18～37）]岁,就诊时高血压6例,血浆肾素活性基础（6479.6±2349.3）ng·L-1·h-1,激发（8446.3±1926.4）ng·L-1·h-1]、血管紧张素Ⅱ基础（114.8±34.2）pg/ml,激发（297.3±87.1）pg/ml]及血浆醛固酮基础（0.35±0.04）nmol/L,激发（0.63±0.18）nmol/L]水平增高6例,低血钾4例。除1例拒行CT检查外,5例由动态增强CT定位。结果保守治疗1例,根治性患肾切除1例,保留肾单位的肾肿瘤切除4例,病理组织学及免疫组化证实肾球旁细胞瘤的诊断。术后随访4～160个月,血压、血钾、肾素、醛固酮均在正常范围内,未见肿瘤复发和转移。结论肾球旁细胞瘤具有高血压、高肾素血症、高醛固酮血症、低血钾的临床特点,动态CT增强扫描有助于定位,免疫组化染色CD34、肌动蛋白（Actin）阳性,细胞角蛋白（CK）阴性是其特征,保留肾单位的肿瘤切除术是有效的治疗方法。

Analysis of 6 cases juxtaglomerular cell tumor of kidney and literature review

Objective To explore the clinical features of juxtaglomerular cell tumor of the kidney and improve its diagnosis and treatment.Methods Six patients（5 males,1 female） of juxtaglomerular cell tumor of the kidney were retrospectively analyzed and relevant literatures were reviewed.The mean age was26.2±5.7（18～37）] years at diagnosis.All six cases suffered from hypertension.The laboratory examinations revealed that,respectively in decubitus and standing position,the plasma renin activity was（6 479.6±2 349.3） ng·L-1·h-1 and（8 446.3±1 926.4） ng·L-1·h-1;angiotensin Ⅱ was（114.8±34.2） pg/ml and（297.3±87.1） pg/ml;aldosterone was（0.35±0.04） nmol/L and（0.63±0.18） nmol/L.Hypokalemia were detected in four cases.Five cases tumor were localized with dynamic enhanced CT scan except one who refused further examination.Results One case received radical nephrectomy and four cases underwent partial nephrectomy.One case adopted conservative treatment.Pathological examination confirmed the final diagnosis of juxtaglomerular cell tumor.The blood pressure,serum potassium,renin and aldosterone maintained in normal range during the following up period（4-160 months）.No tumor progress and metastasis were found.Conclusions The juxtaglomerular cell tumor is featured as the composite clinical symptoms of hypertension,hyperreninemia,hyperaldosteronism,and hypokalemia,The dynamic enhanced CT scan was helpful in tumor localization.The positive immunoreactive for CD34 and Actin,and negative for CK were the characteristics for identification.Nephron-spare surgery is recommended for the treatment.