Endocrine Abstracts (2009) 19 P30

Bilateral testicular adrenal rests in congenital adrenal hyperplasia

V Nayyar, D Bosman & K Laji

Department of Diabetes & Endocrinology, St Richards Hospital, Royal West Sussex NHS trust, Chichester, UK.

Background: Testicular tumours have been described in patients with congenital adrenal hyperplasia (CAH) and are usually associated with 21 hydroxylase deficiency, occasionally it has been reported in patients with 11 hydroxylase deficiency CAH. Poor compliance to adrenocorticotrohphic suppressive therapy can result in the development of testicular tumours which are due to adrenal rest tissue hyperplasia. This is usually benign but can be mistaken for malignant testicular tumours. We present a 32-year-old gentleman who presented with adrenal rest tissue in his testes and nearly escaped bilateral orchidectomy.

Case: We report a 32 year gentleman who was diagnosed as having 11 beta hydroxylase deficiency at the age of 4 years when he presented with precautious puberty. He was doing well since childhood on prednisolone 2.5 mg (am) and 5 mg (pm), until the age of 20 years when his compliance to steroid therapy became irregular and due to social reasons he missed multiple hospital appointments. At the age of 28 he presented to accident and emergency with bilateral testicular swelling. Admitting team suspected testicular malignancy. Ultrasound which showed bilateral testicular tumours and he was referred for urological review and orchidectomy. Luckily the urologist reviewed the past medical history carefully and suspected a link to CAH and thus he narrowly escaped bilateral orchidectomy. The testicular tumours where thought to be adrenal rest tissue due to poor therapeutic compliance with glucocorticoid therapy. Once his compliance improved the testicular tumours reduced in size.

Conclusion: Testicular masses in patients with CAH can be extremely difficult to distinguish between adrenal cortical rest hyperplasia and leydig cell tumours, this may lead to an unnecessary orchidectomy in young men. The most appropriate approach is observation of responses to treatment with glucocoticoids.

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