Reninoma: case report and literature review

Abstract

Reninoma is a tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin. We describe a case of reninoma and provide a review of the literature, with a discussion emphasizing the diagnostic evaluation for such patients. The subject had persistent elevation of both plasma renin activity (PRA) and aldosterone. Imaging studies revealed the presence of a lesion in the renal cortex, which was further identified as a renin-producing lesion via selective venous catheterization following administration of an angiotensin-converting enzyme inhibitor (ACE-I). Following partial nephrectomy, the PRA and plasma aldosterone levels declined rapidly and the blood pressure and potassium supplementation requirements normalized. This case demonstrates the utility of both appropriate imaging studies and selective venous catheterization following provocative administration of an ACE-I for diagnosis.

Juxtaglomerular Cell Tumor that was Preoperatively Diagnosed Using Selective Renal Venous Sampling

“…It has been reported that CT and MRI scans are useful for detecting the presence of JCT (9,(15)(16)(17)(18). However, in our case, the tumor was not detected on CT or MRI performed 10 years before the diagnosis.…”

“…However, the clinical behavior of these lesions can be malignant as a result of severe systemic complications of hypertension. It has been reported that the prevalence of retinopathy, renal insufficiency and left ventricular hypertrophy in cases of JCT is 24%, 3% and 7%, respectively (9). However, in our case, most likely due to the patient's young age and well-controlled blood pressure (resulting from the use of antihypertensive medications), the organ damage was not detected.…”

“…The preoperative diagnosis of JCT is based on the hormonal and radiological findings observed on computed tomography (CT) and/or magnetic resonance imaging (MRI) and lateralization of the plasma renin activity (PRA) on renal venous sampling. However, patients with JCT are occasionally nondiagnostic due to the small size of the tumor and the lack of lateralization of the PRA on renal venous sampling (9). We herein present a case of JCT that was not diagnosed for 17 years most likely due to the small size of the tumor and/or incomplete imaging procedures.…”

“…Patients with JCT are clinically characterized by hypertension with hyperaldosteronism secondary to excessive renin secretion by the tumor. These characteristics usually normalize following removal of the tumor (9). The preoperative diagnosis of JCT is based on the hormonal and radiological findings observed on computed tomography (CT) and/or magnetic resonance imaging (MRI) and lateralization of the plasma renin activity (PRA) on renal venous sampling.…”

An Anusual Cause of Secondary Hypertension in a Young Man

“…The most frequent histological type is renal juxta-glomerular apparatus tumor [4]. However, uterin leiomyosarcoma has already been described in a 60 y/o woman with hypertension and hypokaliemia [1].…”