Testicular adrenal rest tumours in congenital adrenal hyperplasia

Claahsen-van der Grinten, H.L.; Otten, B.J.; Stikkelbroeck, M.M.L.; Sweep, F.C.G.J.; Hermus, A.R.M.M.

doi:10.1016/j.beem.2008.09.007

« Previous Next »Best Practice & Research Clinical Endocrinology & Metabolism
Volume 23, Issue 2 , Pages 209-220, April 2009

Testicular adrenal rest tumours in congenital adrenal hyperplasia

H.L. Claahsen-van der Grinten, MD, PhD
- Department of Paediatric Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands
- Corresponding author. Department of Pediatric Endocrinology, Radboud University Nijmegen Medical Centre, (833), PO Box 9101, 6500 HB Nijmegen, The Netherlands. Tel.: +31-24-3619118; Fax: +31-24-3668532.
B.J. Otten, MD, PhD
- Department of Paediatric Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands
M.M.L. Stikkelbroeck, MD, PhD
- Department of Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands
F.C.G.J. Sweep, MD, PhD
- Department of Chemical Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands
A.R.M.M. Hermus, MD, PhD
- Department of Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands

In adult patients with congenital adrenal hyperplasia (CAH) the presence of testicular adrenal rest tumours (TART) is an important cause of gonadal dysfunction and infertility. In the last decade several papers have focused on the origin and pathogenesis of these tumours. In this paper we review the embryological, histological, biochemical and clinical features of TART and discuss the treatment options. Furthermore, we propose a new five-stage classification of TART, based on sonographic, clinical and biochemical parameters, that may lead to a better follow up and treatment of patients with TART.

Keywords: congenital adrenal hyperplasia, testicular adrenal rest tumours, infertility