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June 10, 1996

Prevalence of Adrenal and Extra-adrenal Conn Syndrome in Hypertensive Patients

Author Affiliations

From the Hypertension and Nephrology Unit, the First Department of Medicine, University Hospital, Mainz (Dr Abdelhamid), and Deutsche Klinik für Diagnostik, Wiesbaden (Drs Müller-Lobeck, Bönhof, Walb, and Röckel); and the Institute of Pathology, University of Homburg/Saar (Drs Pahl and Remberger), Germany.

Arch Intern Med. 1996;156(11):1190-1195. doi:10.1001/archinte.1996.00440100086010

Background:  Primary aldosteronism (PA) is caused by an adrenal aldosterone-producing tumor (A-APT) or adrenal hyperplasia. An extra-adrenal APT (E-APT) as a cause of PA has been reported in 5 cases. Autopsy studies show a high incidence of ectopic adrenocortical tissue. We did a prospective study of the prevalence of A-APTs and E-APTs and the biochemical features of E-APTs in patients with PA.

Methods:  Hypertensive patients (N=3900) referred to our unit were screened for PA by measuring renin activity, urinary aldosterone-18-glucuronide, tetrahydroaldoster one, and 18-hydroxycorticosterone (18-OH-B). Primary aldosteronism was found in 257 cases. The differentiation between A-APTs and adrenal hyperplasia was based on the results of postural response of renin, plasma aldosterone, 18-OH-B, computed tomography, isotope scanning, or adrenal venous aldosterone. Ultrasound examination of the abdomen was used to screen for E-APT.

Results:  The cause of PA was bilateral adrenal hyperplasia in 101 cases, unilateral adrenal hyperplasia in 2, an A-APT in 146, and an E-APT in 1. The site of aldosterone production was uncertain in 7 patients who had normal adrenal glands on computed tomography but refused to undergo isotopic scanning and adrenal venous catheterization. Ultrasound examination disclosed normal retroperitoneum in 4 of the 7 cases but could not rule out E-APT in 3 cases. The biochemical features of the patient with the E-APT were similar to classic A-APT, with low renin, high aldosterone, and high 18-OH-B values without appropriate response to posture or to short-term volume expansion. The excision of the E-APT in the right kidney resulted in normalization of blood pressure and renin, aldosterone, and 18-OH-B levels.

Conclusion:  Although E-APT is rare, it should be considered in the interests of specific therapy for PA because aldosterone-secreting malignant ovarian tumors also have been reported.(Arch Intern Med. 1996;156:1190-1195)