Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia.
Faculty of Medicine, University of Adelaide, Adelaide, South Autralia 5000, Australia.
J Clin Endocrinol Metab. 2024 Jan 18;109(2):e809-e816. doi: 10.1210/clinem/dgad516.
A reninoma is a functional tumor of afferent arteriolar juxtaglomerular cells that secretes the enzyme renin, leading to hyperactivation of the renin-angiotensin-aldosterone system. Reninoma is a potentially curable cause of pathological secondary hyperaldosteronism that results in often severe hypertension and hypokalemia. The lack of suppression of plasma renin contrasts sharply with the much more common primary aldosteronism, but diagnosis is often prompted by screening for that condition. The major differential diagnosis of reninoma is renovascular hypertension. Fewer than 200 cases of reninoma have been described. Reninomas have been reported across a broad demographic but have a 2:1 predilection for women, often of childbearing age. Aldosterone receptor blockade, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers offer effective medical management but are contraindicated in pregnancy, so surgical curative resection is ideal. The current optimal imaging and biochemical workup of reninoma and management approach (ideally, tumor excision with subtotal renal resection) are described.
肾素瘤是一种功能性肿瘤，来源于入球小动脉近肾小球细胞，分泌肾素，导致肾素-血管紧张素-醛固酮系统过度激活。肾素瘤是一种潜在可治愈的继发生理性醛固酮增多症的病因，常导致严重的高血压和低钾血症。与更为常见的原发性醛固酮增多症不同，肾素瘤患者的血浆肾素水平无抑制，但其诊断通常是在原发性醛固酮增多症筛查时发现的。肾素瘤的主要鉴别诊断是肾血管性高血压。目前已描述的肾素瘤病例不足 200 例。肾素瘤报告见于广泛的人群，但女性发病率为 2 比 1，且多为育龄妇女。醛固酮受体阻滞剂、血管紧张素转换酶抑制剂或血管紧张素受体阻滞剂可有效进行药物治疗，但在怀孕期间禁用，因此手术切除是理想的治疗方法。本文介绍了目前肾素瘤的最佳影像学和生化检查及治疗方法（理想情况下，采用肿瘤切除术加部分肾切除术）。
