Department of Endocrinology and Metabolism, Tianjin Medical University General Hospital, Tianjin, China.
Department of Clinical Medicine, Tianjin Medical University, Tianjin, China.
BMC Endocr Disord. 2022 Mar 18;22(1):71. doi: 10.1186/s12902-022-00977-w.
Reninoma is a rare, benign renal neoplasm. Typical clinical features include severe hypertension, secondary hyperaldosteronism, hypokalaemia and metabolic alkalosis caused by the overproduction of renin.
A 25-year-old lean Chinese woman with no family history of hypertension was hospitalized for stage 1 hypertension that gradually developed over two years. Endocrine investigation showed hyperreninemia without hyperaldosteronism and hypokalaemia. Interestingly, although the patient had an elevated plasma renin concentration (PRC), her plasma renin activity (PRA) was in the normal range. Abdominal contrast-enhanced computed tomography (CT) scanning revealed a solid, low-density, renal cortical mass with delayed enhancement. Selective renal vein sampling (SRVS) was performed, and a lateralization of the renin secretion from the left kidney was found. Enucleation of the tumour led to a rapid remission of hypertension and hyperreninemia. Based on pathological findings, the patient was diagnosed with reninoma. Immunohistochemical staining of the tumour was positive for Renin, CD34, Vimentin, and synaptophysin (Syn) and negative for somatostatin receptor 2 (SSTR2) and chromogranin A (CgA).
Reninoma can present as mild hypertension without hyperaldosteronism and hypokalaemia. The clinical features of reninoma may depend on the degree of activation of the renin-angiotensin-aldosterone system (RAAS). PRC should be incorporated in the differential diagnosis of secondary hypertension.
肾素瘤是一种罕见的良性肾肿瘤。其典型的临床特征包括严重的高血压、继发醛固酮增多症、由于肾素过度产生导致的低钾血症和代谢性碱中毒。
一位 25 岁的瘦弱中国女性，无高血压家族史，因逐渐发展的 2 级高血压入院。内分泌检查显示高肾素血症，但无醛固酮增多症和低钾血症。有趣的是，尽管患者的血浆肾素浓度（PRC）升高，但她的血浆肾素活性（PRA）处于正常范围内。腹部增强 CT 扫描显示一个实性、低密度、肾皮质肿块，增强后延迟强化。进行了选择性肾静脉取样（SRVS），发现左肾的肾素分泌偏向。肿瘤剜除术导致高血压和高肾素血症迅速缓解。根据病理发现，该患者被诊断为肾素瘤。肿瘤的免疫组化染色结果为 Renin、CD34、Vimentin 和 synaptophysin（Syn）阳性，somatostatin receptor 2（SSTR2）和 chromogranin A（CgA）阴性。
肾素瘤可表现为轻度高血压，无醛固酮增多症和低钾血症。肾素瘤的临床特征可能取决于肾素-血管紧张素-醛固酮系统（RAAS）的激活程度。PRC 应纳入继发性高血压的鉴别诊断。
