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Testicular Adrenal Rest Tumors in Adult Males with Congenital Adrenal Hyperplasia: Evaluation of Pituitary-Gonadal Function before and after Successful Testis-Sparing Surgery in Eight Patients

Departments of Paediatric Endocrinology (H.L.C.-v.d.G., B.J.O.), Radiology (S.T.), Urology (E.J.H.M.), Pathology (C.H.-v.d.K.), Chemical Endocrinology (F.C.G.J.S.), and Endocrinology (A.R.M.M.H.), Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands

Address all correspondence and requests for reprints to: H. L. Claahsen-van der Grinten, M.D., Radboud University Nijmegen Medical Centre, Department of Paediatric Endocrinology (833), P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. 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: The aim of this study was to evaluate tumor volume, symptoms, and pituitary-gonadal function in male patients with CAH caused by 21-hydroxylase deficiency and bilateral TART before and after testis-sparing surgery.

Setting: This study was conducted at Radboud University Nijmegen Medical Centre in The Netherlands.

Patients: Eight adult male CAH patients with bilateral TART and infertility were included.

Interventions: Evaluation of testicular magnetic resonance imaging, symptoms, fasting serum concentrations of ACTH, LH, FSH, inhibin B, 17-OH progesterone, androstenedione, testosterone, and estrone, and semen analysis (six of eight patients) was performed before and 6 and 22 months after testis-sparing surgery.

Main Outcome Measures: The main outcome measures were absence of residual tumor and improvement of symptoms and pituitary-gonadal function.

Results: Residual tumors were not found on any of the patients’ magnetic resonance imaging 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 (five patients) or oligospermia (one 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.

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