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This Article Full Text Full Text (PDF) All Versions of this Article: EJE-09-0576v1 EJE-09-0576v2 162/2/213    most recent Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Dennedy, M. C. Articles by McKenna, T J. Search for Related Content PubMed PubMed Citation Articles by Dennedy, M. C. Articles by McKenna, T J.

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

Department of Endocrinology and Diabetes Mellitus, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland
¹ Department of Endocrinology, Beaumont Hospital, Beaumont Road, Dublin 9, Ireland

(Correspondence should be addressed to T J McKenna; Email: mckennatj{at}gmail.com)

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 µg i.v., to screen for CAH, which is present in 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.