肾球旁细胞器瘤的临床分型与诊治探讨

Classification and treatment of juxtaglomerular cell tumor of the kidney

目的介绍一种新的肾球旁细胞器瘤临床分型方法，并对其临床诊治进行探讨。方法收集肾球旁细胞器瘤患者5例，结合PubMed搜索到较大的系列病例报道66例，对71例病例资料进行分型。对患者的临床表现、实验室检查和影像学检查进行分析。临床资料包括血压；实验室检查包括血钾、血浆肾素和醛固酮、静脉分段取血检测；影像学检查包括超声、CT、IVU、选择性肾血管造影等。结果依据血压和血钾水平，71例肾球旁细胞器瘤可分为3种类型：典型、非典型和静止型。57例典型病例特点：高血压、低血钾、高醛固酮和低肾素；12例非典型病例的特点：高血压、正常血钾；2例静止型病例特点：正常血压和血钾。对于具有高血压、低血钾、高肾素和高醛固酮的患者，应考虑典型肾球旁细胞器瘤的可能；对于高血压合并肾肿瘤的患者，应考虑非典型球旁细胞器瘤的可能；对于单纯肾脏肿瘤，应考虑静止型肾球旁细胞器瘤的可能。结论肾球旁细胞器瘤划分为典型、非典型和静止型3类的依据是血压和血钾。肾球旁细胞器瘤代表了一种可以手术纠正的高血压病变，肾肿瘤鉴别诊断中应考虑肾球旁细胞器瘤的可能。保留肾单位手术是首选治疗方法。

Objective To study the classification and treatment of juxtaglomerular cell tumor of the kidney. Methods Five cases of juxtaglomerular cell tumors of the kidney were diagnosed and treated surgically in our hospital during the last 4 years. Seven large series of cases report of juxtaglo- merular cell tumors have been published in the Pubmed. These series of cases report plus 1 case report of static juxtaglomerular cell tumor were incorporated into a review of 71 cases previously published in English literature. The clinical presentation, laboratory examination and imaging study were summarized. The clinical presentation including blood pressure, the laboratory examinations including kalium, plasma renin activity and aldosterone, and renal venous sampling for renin assay, and the imaging study including ultrasonography, CT, excretory urography, and selective renal angiography were studied retrospectively. Results The 71 cases juxtaglomerular cell tumors could be classified into 3 types, that is, typical type, atypical type and static type. The 57 typical cases had the typical characteristics of hypertension, hyperaldosteronism, and hypokalemia secondary to tumor renin secretion. The 12 atypical cases had hypertension with normal kalium, and the 2 static cases had normal blood pressure and kalium. Typical juxtaglomerular cell tumor of the kidney should be considered in hypertensive patients with secondary aldosteronism. Atypical juxtaglomerular cell tumor of the kidney should be considered in patients with hypertension and renal tumor. Static juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor. Conclusions The classification of typical, atypical and static juxtaglomerular cell tumors depends on blood pressure and serum kalium. As a surgically corrected disease, juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor, and nephron sparing surgery is the first choice.