Authors: P J Guillausseau C Boitard Y Le Charpentier L Cedard K Nahoul C Blacker E Kaloustian F CourtalhacKaloustian C Dubost J Lubetzki
Publish Date: 2014/03/27
Volume: 10, Issue: 6, Pages: 593-599
Abstract
In a 41yearold hirsute woman severe hypercalcemia led to the discovery of hyperparathyroidism related to the involvement hyperplasia/or adenoma of the 4 parathyroid glands Plasma and urinary DHA plasma DHAsulfate and A 5 steroid precursors were elevated Steroid hormone hypersecretion was stimulated by hCG and ACTH and exhibited a paradoxical rise during dexamethasone administration Computerized tomography scanning as well as arteriography disclosed bilateral adrenal hyperplasia and left adrenal adenoma Bilateral adrenal vein catheterization indicated a left/right gradient for Δ 5 steroids and Δ 5 steroid sulfates At surgery a left brown adrenal encapsulated adenoma was removed with a hyperplastic adrenal gland Results of in vitro studies adrenal steroid content and incubation together with postadrenalectomy hormonal results suggest that the left brown adrenal adenoma was the main source of excessive androgen production The infrequent association of an androgenproducing adrenal adenoma with hyperparathyroidism raises the hypothesis of multiple endocrine neoplasia syndrome However evidence for this diagnosis is lacking in the absence of other glandular involvement and of family history
Keywords: