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This version published online on November 7, 2006
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2006-1311
A more recent version of this article appeared on February 1, 2007
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Submitted on June 19, 2006
Accepted on November 1, 2006

Testicular adrenal rest tumours in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testis-sparing surgery in 8 patients

Hedi L. Claahsen-van der Grinten*, Barto J. Otten, Satoru Takahashi, Eric J.H. Meuleman, Christina Hulsbergen-van de Kaa, Fred C.G.J. Sweep, and Ad R.M.M. Hermus

Departments of Paediatric Endocrinology, Radiology, Urology, Pathology, Chemical Endocrinologyand Endocrinology, Radboud University Nijmegen Medical Centre, The Netherlands (*Present address: Department of Urology, VU University Medical Centre Amsterdam, The Netherlands)

* To whom correspondence should be addressed. E-mail: H.Claahsen{at}cukz.umcn.nl.

Context: In male patients with congenital adrenal hyperplasia (CAH) testicular adrenal rest tumors (TART) are frequently present. These tumors can interfere with testicular function. Intensifying glucocorticoid therapy does not always lead to tumor regression and improvement of testicular function. Recently testis-sparing surgery was introduced for treatment of TART.

Objective: Evaluation of tumor volume, symptoms and pituitary-gonadal function in male patients with CAH due to 21-hydroxylase deficiency and bilateral TART before and after testis-sparing surgery.

Setting: Radboud University Nijmegen Medical Centre, The Netherlands

Patients: 8 adult male CAH patients with bilateral TART and infertility.

Interventions: Evaluation of testicular MRI, symptoms, fasting serum concentrations of ACTH, LH, FSH, inhibin B, 17-OH progesterone, androstenedione, testosterone and oestron, and semen analysis (6 of 8 patients) before, and 6 and 22 months after testis-sparing surgery.

Main outcome measures: Absence of residual tumor. Improvement of symptoms and pituitary-gonadal function.

Results: In none of the patients residual tumor was found on MRI after surgery. Two patients reported testicular pain and discomfort that disappeared after surgery. Parameters of pituitary-gonadal function did not improve after surgery: Semen analysis showed azoospermia (5 patients) or oligospermia (1 patient) without improvement and all patients had persistently low inhibin B concentrations.

Conclusion: Testis-sparing surgery did not improve pituitary-gonadal function despite successful removal of the tumors. Further studies are needed to investigate whether surgery at an earlier stage in the natural history of TART can prevent permanent testicular damage.
Key words: congenital adrenal hyperplasia • testicular adrenal rest tumour • infertility • testis-sparing surgery




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