Renin-Secreting Clear Cell Carcinoma of the Kidney

Two types of renal tumors (juxtaglomerular cell¹⁻⁷and Wilm⁸,⁹) have been reported as curable forms of hypertension associated with overproduction of renin by the tumor. Patients with this disorder have had a syndrome of severe hypertension, hypokalemia, increased peripheral vein renin activity, and secondary aldosteronism, all of which disappear following removal of the tumor. To our knowledge, this report describes the first case of hypertension associated with renin production by a clear cell carcinoma of the kidney that was cured after nephrectomy. Furthermore, the characteristic findings in previously reported renin-secreting tumors of severe hypertension, hypokalemia, elevated plasma renin activity (PRA), and secondary aldosteronism were not present. PATIENT SUMMARY A 29-year-old woman was referred to Vanderbilt University Hospital with the provisional diagnosis of Cushing syndrome, because of a two-year history of hypertension, obesity, and hirsutism. Her menses were regular from age 12 years until one year prior to admission