Androgen producing adrenal adenoma. Report on a case associated with hyperparathyroidism.
In a 41-year-old 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 DHA-sulfate and delta 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 delta 5 steroids and delta 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 androgen-producing 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.
D000236:Adenoma / Q000150:complications / Q000378:metabolism*; D000305:Adrenal Cortex Hormones / Q000097:blood; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000378:metabolism*; D000311:Adrenal Glands / Q000473:pathology / Q000601:surgery; D000328:Adult; D000728:Androgens / Q000096:biosynthesis*; D000792:Angiography; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000150:complications*; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis; D010280:Parathyroid Glands / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed
D000305:Adrenal Cortex Hormones; D000728:Androgens