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Sex-hormone Producing Adrenal Gland Tumors
SEX-HORMONE PRODUCING ADRENAL GLAND TUMORS
Adrenal androgen-secreting tumors (virilizing adrenal tumors), adenomas and adenocarcinomas are rare and their clinical manifestations vary depending on age at onset , accounting for only 0.2% of the causes of androgen excess . Serum androgen levels are below normal in patients with benign cortisol-secreting adrenocortical adenomas, owing to ACTH suppression. Associated androgen secretion usually is highly suggestive of malignancy. Virilizing adrenocortical adenoma is a rare cause of androgen excess in young women presented as hirsutism, acne, and oligo/amenorrhea. Testosterone is considered to be a major steroid hormone produced by these tumors. The plasma testosterone concentration is high in such virilizing patients [3, 4].
The hormonal status of adenomas is usually characterised by a predominant increase in plasma androstenedione (> 600, ng/dl) and testosterone (> 200 ng/dl), whereas carcinomas present a predominant hypersecretion of dehydroepiandrosterone (> 1200 ng/dl) and its sulphate (> 700 micrograms/dl) .
Differential diagnosis must be made with androgen secreting tumors of the ovaries and, in males, with the various forms of precocious puberty, including endocrine tumors of the testicle, as well as congenital adrenogenital syndrome . Treatment is surgical removal of the adrenal gland.
- Sciarra F, Tosti-Croce C, Toscano V. Androgen-secreting adrenal tumors. Minerva Endocrinol. 1995 Mar;20(1):63-8.
- d'Alva CB, Abiven-Lepage G, Viallon V, Groussin L, Dugue MA, Bertagna X, Bertherat J. Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non-tumoral causes of androgen excess. Eur J Endocrinol. 2008 Nov;159(5):641-7.
- Gabrilove JL, Seman AT, Sabet R, Mitty HA, Nicolis GL (1981) Virilizing adrenal adenoma with studies on the steroid content of the adrenal venous effluent and a review of the literature. Endocrine Rev 2: 462-470.
- Baulieu E, Wallace E, Lieberman S (1963) The conversion in vitro of i\5-androstene-3/3, 17/3diol-7a3H to testosterone 17a3H by human adrenal and placental tissue. J Biol Chem 238:1316-1319.