Juxtaglomerular cell tumor of the kidney--a new classification scheme

To introduce a new classification scheme of juxtaglomerular cell tumor (JCT) of the kidney for differential diagnosis of hypertension and renal cell carcinoma.

Five cases of JCT have been diagnosed and treated surgically in our hospital during the last 4 years. Through a search in PubMed, we incorporated 7 large series of case reports of JCT into a review of 71 cases previously published in the literature. Clinical presentations (blood pressure), laboratory examinations [serum potassium, plasma renin activity (PRA), aldosterone (ALD), and renal venous sampling for renin assay], and imaging examinations [ultrasonography, computerized tomography (CT), excretory urography, and selective renal angiography] were summarized.

The 71 cases of JCTs can be classified into 3 types, which are typical type, atypical type, and non-functioning type. The 57 typical cases had the typical characteristics of hypertension, hypokalemia, hyperaldosteronism, and high renin. The 12 atypical cases had hypertension with normal potassium levels, and the 2 non-functioning cases had normal blood pressure and normal potassium levels.

The classification of typical, atypical, and non-functioning JCTs depends on blood pressure and serum potassium. JCT of the kidney should be considered in patients with hypertension and renal tumor, and nephron-sparing surgery is the first choice.