Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases

Abstract

A 15-year-old girl with severe high renin hypertension caused by a juxtaglomerular cell tumor (JCT) was successfully treated with the calcium channel blocker nifedipine until surgical removal effected a permanent cure. This case was incorporated into a review of the 37 cases previously published. Comparison of the children and adolescents with the adult population showed that the features of JCT were similar in the two groups except for the average duration of symptoms prior to diagnosis (pediatric group 2.6 years vs. 6.0 years for the adult group). Analysis of all 38 cases demonstrated the following: 1. Teenagers constituted the largest single population with JCT (39%) and approximately two-thirds of the entire population were female. 2. Many patients failed to show persistent hypokalemia despite high plasma renin activity and secondary hyperaldosteronism. 3. Renal angiography was initially negative in more than half the cases. 4. Renal vein renin failed to show lateralization to the affected kidney in 52% of the cases. 5. Computerized tomography demonstrated a renal mass in all of the cases in which it was performed, even when other imaging studies were negative. 6. Calcium channel blockers may evolve as the preferred treatment for the high renin hypertension of JCT.

Loss of chromosomes 9 and 11 may be recurrent chromosome imbalances in juxtaglomerular cell tumors

“…Less than 100 cases have been published, mainly as isolated case reports or small series. JGCTs are twice more common in women than in men and develop mostly in the second and third decades of life [2]. Clinically, hypertension is present in almost all patients because of renin secretion by the tumor cells.…”

Chromosome abnormalities in juxtaglomerular cell tumors

“…After overnight fixation in McDowell's fixative, pH 7.4 (4% formaldehyde, 1% glutaraldehyde in a 320 mOsm phosphate buffer), finely cut tumor pieces were postfixed in 1% OsO 4 , dehydrated in a graded series of ethanol washes, critical point dried in a dryer from Balzer Union (Bal-Tec, Lichtenstein), mounted on aluminum stubs before coating with gold using a Polaron SEM Coating Unit E5000 (Polaron, Waterford, England), and embedded in ethylether/araldite (Serva, Heidelberg, Germany). The sections were evaluated in a Jeol 1010 transmission electron microscope (Jeol, Tokyo, Japan).…”

The usefulness of renal vein renin studies in hypertensive children: a 25-year experience

“…However, some other investigators, with careful inclusion criteria such as severe and refractory hypertension, unilateral renal artery stenosis with an estimated lumen obstruction to less than a quarter and exclusion criteria such as extreme age, disseminated severe atherosclerosis and reduced renal function, have shown that RVR ratios can be highly predictable of successful surgical outcome [13,38]. Their value in the recognition of renin-secreting tumours, however, is marginal [39,40].…”