A 40-year-old woman with hypertension (179/100) and a massive renal arteriovenous fistula involving most of the right kidney is reported. A 131I hippuran renogram, split urinary function studies and renal vein renins were normal. The renal arteriovenous fistula was removed by total nephrectomy and blood pressure returned to normal. A renal cell carcinoma was found within the fistula. This case confirms the association of renal cell carcinoma with renal arteriovenous fistulas and demonstrates the potential curative effects of nephrectomy for the hypertension associated with and demonstrates the potential curative effects of nephrectomy for the hypertension associated with it. The normal split function studies, renogram and renal vein renins do not support the concept of secondary hyperreninemia as a causative factor in the hypertension seen with renal arteriovenous fistulas.
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