Prevalence of adrenal and extra-adrenal Conn syndrome in hypertensive patients.
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, tetrahydroaldosterone, 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.


CONCLUSIONS
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.
Abdelhamid
S
S
First Department of Medicine, University Hospital, Mainz, Germany.
Müller-Lobeck
H
H
Pahl
S
S
Remberger
K
K
Bönhof
J A
JA
Walb
D
D
Röckel
A
A
D000306:Adrenal Cortex Neoplasms / Q000097:blood / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D000450:Aldosterone / Q000097:blood; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006929:Hyperaldosteronism / Q000209:etiology* / Q000378:metabolism; D006973:Hypertension / Q000209:etiology* / Q000378:metabolism; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies
D000450:Aldosterone