肾素分泌瘤9例临床特征分析并文献复习

Analysis of clinical features of 9 patients with renin secreting tumor and literature review

目的分析9例肾素分泌瘤患者的临床特征并进行相关文献复习,以提高对该病的认识及诊疗水平。方法收集解放军总医院第一医学中心2010年1月-2020年2月收治的9例肾素分泌瘤患者的临床资料、诊治情况、病理和免疫组化染色结果进行回顾性分析。检索2001-2020年的相关数据库,结合文献报道的142例患者分析肾素分泌瘤的临床特点。结果9例患者平均年龄22.6岁,均表现为高血压,7例为低钾血症。所有患者血浆肾素水平均明显高于正常,醛固酮水平高于正常参考区间中位数。术后病理证实均为肾球旁细胞瘤。免疫组化染色结果显示肿瘤细胞肾素、波形蛋白和CD34均呈阳性,部分肿瘤细胞平滑肌肌动蛋白(SMA)和突触素(Syn)呈阳性,S-100蛋白(S-100)和嗜铬素A(CgA)均呈阴性。术后所有患者血压和血钾水平恢复正常。2001-2020年共报道我国肾素分泌瘤患者142例,首发症状均为高血压,87例为低钾血症,93例血肾素与醛固酮水平增高,随访所有患者均未出现复发及转移情况。结论肾素分泌瘤多为肾良性肿瘤,临床表现为高血压、低血钾、高肾素、高醛固酮血症的特点,该病可手术治疗,一般预后良好。

Objective To analyze the clinical features of 9 patients with renin secreting tumor and review the related literature in order to improve the understanding,diagnosis and treatment of the disease.Methods The clinical data,diagnosis,treatment,pathological and immunohistochemical profiles of 9 patients with renin secreting tumor admitted in the First Medical Center of Chinese PLA General Hospital from January 2010 to February 2020 were retrospectively analyzed.The literature was searched by retrieving the related database from 2001 to 2020,combined with 142 similar cases collected by searching the database to analyze the clinical features of patients with renin secreting tumor.Results All the 9 patients with an average age of 22.6 years presented with hypertension,and 7 of the 9 with hypokalemia.The plasma renin levels in all the patients were significantly higher than normal range,and the plasma aldosterone levels were above the median normal reference interval.Postoperative pathological examination of all cases confirmed juxtaglomerular cell tumor.Immunohistochemical staining showed that all the tumor cell renin,vimentin and CD34 were positive,some neoplastic cells were positive for smooth muscle actin(SMA)and synaptophysin(Syn),and negative for S-100 protein(S-100)and chromogranin A(CgA).The blood pressure and serum potassium level returned to normal range in all patients after surgery.A total of 142 patients with renin secreting tumor have been reported in China from 2001 to 2020.Hypertension was the initial symptom of all the patients.Hypokalemia occurred in 87 of 142 patients.Plasma renin and aldosterone levels were above the normal range in 93 patients.No recurrence or metastasis occurred in all patients after surgery during follow-up.Conclusions Renin secreting tumor mostly is characterized as benign renal tumor with hypertension,hypokalemia,hyperreninemia and hyperaldosteronism.Surgery is preferred in treatment of this tumor for a good prognosis.