Unilateral renal hypertension due to angiomatous tumor of juxtaglomerular cell origin: report of a case

Abstract

An extremely rare case of unilateral renal hypertension due to angiomatous tumor of juxtaglomerular cell origin is presented.A 23-year-old woman was admitted to Niigata University Hospital on April 7, 1967 with complaint of headache, stiff shoulder and hypertension. Physical examination revealed almost healthy except for hypertension, systolic murmur at the cardiac apex and angiosclerosis retinae on the fundus appearance. Blood chemistry showed hypokalemia, but there was neither fatigue, nor polydipsia, nor polyuria. Urinalysis, cystoscopic examination and renal function study were within normal limits. Aldosterone secretion rate was moderately elevated, but plasma renin activity was extremely high. Intravenous pyelography showed a filling defect in the upper part of left renal pelvis, and contrast medium distribution on the aortography was slightly faint in the upperr part of the left kidney. Left nephrectomy was performed on May 15 with a diagnosis of unilateral renal hypertension with secondary aldosteronism.On cut section of the specimen, a well encapsulated, round and soft tumor (2cm in diameter) was found in the middle portion of the renal parenchyma, just adjacent to the renal pelvis. Pathological report reported that the tumor was vascular in nature and had a similar appearance to hemangiopericytoma. Bowie's staining revealed abundant blue-purplish granules in the cytoplasm of main cellular elements of the tumor. These granules were thought to be identical with the juxtaglomerular cell granules which were characteristically found in cases of renovascular hypertension. The tumor can be designated as “juxtaglomerular cell tumor”.Following the surgery the blood pressure have been measured normal. Postoperatively, plasma renin activity was also decreased to the normal level. Discharge on June 10, 1967 with definite improvement.