Pathologic Characterization of a Renin-Secreting Juxtaglomerular Cell Tumor in a Child and Review of the Pediatric Literature

Abstract

We describe a rare case of renal hypertension in a 15-year-old caused by juxtaglomerular cell tumor and compare our findings with those of 20 children reported in the literature. These tumors are usually encapsulated and composed microscopically of polyhedral cells with bland nuclei separated by fibrovascular septa. Characteristic renin granules can be demonstrated by Bowie's stain or electron microscopy. These tumors are benign. Pathologists should recognize the morphologic characteristics of these tumors when dealing with renin-producing neoplasia.

Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

“…This tumor would also likely have been missed on MRI because of its small size. Conventional angiography, which was briefly contemplated for the patient, would have also missed the tumor (18). …”

MR findings of renin-secreting tumor: a case report

“…Because enucleation is usually effective for renin-secreting tumors, preoperarive localization and lateralization are very important. Reninsecreting tumors are usually small, peripherally located, have an echogenicity similar to normal renal parenchyma, and are hypovascular or avascular, which make visualization difficult during intravenous urography, sonography, and angiography [1,2,4,5,8,10]. Because of the peripheral location of the tumor and the possibility of tumor venous drainage via the pericapsular vein, the location of the sampling catheter is irrelevant to the tumor location; sampling via the main renal vein may not always reveal significant lateralization of PRA [5].…”

“…The renin-secreting tumor, also known as the juxtaglomerular cell tumor or hemangiopericytoma, is a rare benign tumor of the kidney [8]. It is often found in young adults.…”

“…At least 48 well-documented cases have been reported in the English literature [1][2][3][4][5][6][7][8][9] since the first case was described in 1967 [1]. But the magnetic resonance (MR) findings of this rare disease entity have been described in only one study [9].…”

Juxtaglomerulärer Zelltumor (Reninom) als Ursache einer schweren juvenilen Hypertonie

“…Die Diagnose "reninproduzierender juxtaglomerulärer Tumor" wird durch den typischen histologischen Befund (Übersicht bei [1]) bestätigt: Verbände kleiner polygonaler Zellen, zum Teil mit hyperchromen Kernen, umgeben von fibrösen Septen mit zahlreichen kleinen Arteriolen und Kapillaren. In den Zellen können charakteristische (Renin enthaltende) Granula nachgewiesen werden.…”