Publication

Investigation of patients with atypical or severe hyperandrogenaemia including androgen-secreting ovarian teratoma.

Dennedy, Michael Conall
Smith, Diarmuid
O'Shea, Donal
McKenna, T Joseph
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Date
2012-02-01T10:34:03Z
Date Submitted
Keywords
Other Subjects
Subject Mesh
Androgens/secretion
Female
Humans
Hyperandrogenism/*diagnosis/*etiology
Ovarian Neoplasms/*complications/*diagnosis/secretion
Severity of Illness Index
Teratoma/*complications/*diagnosis/secretion
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Abstract
Approximately 7% of women of reproductive age manifest polycystic ovary syndrome (PCOS) and <0.5% have other causes of hyperandrogenism including congenital adrenal hyperplasia (CAH), androgen-secreting tumour of an ovary or an adrenal gland, Cushing's syndrome or hyperthecosis. The presence of features atypical of PCOS should prompt more extensive evaluation than that usually undertaken. Features atypical of PCOS include the onset of symptoms outside the decade of 15-25 years, rapid progression of symptoms, the development of virilization and a serum testosterone concentration in excess of twice the upper limit of the reference range. Ethnic background, family history and specific clinical findings, e.g. Cushingoid appearance, may inform a focused investigation. Otherwise, patients should have measurement of 17-hydroxyprogesterone (17-OHP) under basal conditions ideally in the early morning, and if abnormal, they should have measurement of 17-OHP one hour after the administration of synthetic ACTH, 250 microg i.v., to screen for CAH, which is present in approximately 2% of hyperandrogenic patients. The overnight cortisol suppression test employing 1 mg dexamethasone at midnight is a sensitive test for Cushing's syndrome. Coronal tomographic (CT) scanning of the adrenals and transvaginal ultrasonography of the ovaries are the investigations of choice when screening for tumours in these organs. Less frequently required is catheterization and sampling from both adrenal and ovarian veins, which is a technically demanding procedure with potential complications which may provide definitive diagnostic information not available from other investigations. Illustrative case reports highlight some complexities in the investigation of hyperandrogenic patients presenting with features atypical of PCOS and include only the ninth case report of an androgen-secreting ovarian teratoma.
Language
eng
ISSN
1479-683X (Electronic)
0804-4643 (Linking)
eISSN
ISBN
DOI
10.1530/EJE-09-0576
PMID
19906851
PMCID
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