Renin-secreting juxtaglomerular-cell tumour.

Abstract

Selective renal arteriography demonstrated an 8 mm renin secreting renal juxtaglomerular-cell tumour in an eight year old girl presenting with hypertension. This is an uncommon tumour so far only reported in patients under 40 years. The largest tumour has been 4 cm in diameter. On selective renal arteriography the tumour appears as an area of relative translucency during the nephrogram phase, often bulging the cortex, and surrounded by a more dense rim of compressed renal parenchyma. A few dilated tortuous arteries may also be present. The clinical, radiological and pathological similarities between this case and those previously described help to establish a syndrome, sometimes referred to as primary reninism, in which hypertension is associated with primary over-secretion of renin by a renal tumour. The diagnosis depends on simultaneous bilateral renal vein catheterization and selective renal arteriography. A factor of crucial importance is the elevation of venous plasma renin from the affected kidney.