CASE REPORT Tug˘rul Tiryaki Æ Zehra Aycan Æ Sema Hu¨ cu¨ menog˘lu Halil Atayurt Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia Accepted: 9 August 2005 / Published online: 23 September 2005 Ó Springer-Verlag 2005 Abstract The association between testicular tumors/ nodules and congenital adrenal hyperplasia (CAH) has been recognized for many years. Tumors are considered to be an aberrant adrenal tissue that has descended with the testes and has become hyperplastic due to ACTH stimulation. The recommended treatment consists of increasing the glucocorticoid dose to suppress ACTH secretions. If the testicular size is not reduced after suppression therapy or a side effect of glucocorticoid dose is noted, surgical intervention should be consid- ered. We diagnosed steroid unresponsive testicular tu- mors of the CAH in two patients who were treated by testicular sparing tumor enucleation. We believe that testis sparing surgery is the procedure of choice for all patients with testicular adrenal rest tumor, since it maximizes future fertility potential. Keywords Congenital adrenal hyperplasia Æ Testicular adrenal rest tumors Introduction Testicular adrenal rest tumors are increasingly reported in male patients with congenital adrenal hyperplasia (CAH) [1, 2]. Testicular masses may create diagnostic difficulties clinically and pathologically. The exact cellular origin of this tumor and the management of these patients is still controversial. When the presence of the adrenogenital syndrome is not appreciated, the mass is usually assumed to be a neoplasm and an orchidectomy is performed. The lesion may be misin- terpreted as a Leydig cell tumor on pathological examination. We have presented two patients with congenital adrenal hyperplasia whose testicular tumors did not regress with suppression therapy and discussed briefly the management of CAH patients with testicular enlargement. Case report Case 1 A 17 year-old boy was presented with bilateral testicular swelling of 1 month duration. A diagnosis of 11-hydroxylase deficiency had been made at the age of 15 months when he presented with dehydration, and electrolyte abnormalities. Physical examination dis- closed bilateral nodular testes. Bilateral testes volume were over 25 ml and poor compliance of suppressive therapy was noted. Scrotal ultrasonography showed heterogenic testis parenchyma and multiple different sized hypoechoic nodules. On magnetic resonance imaging (MRI), a lesion filling the scrotum with dimensions of 10·8·8 cm was noted (Fig. 1). On T1a and T2a images, the mass was hypointense and was described as a rest tumor. Azospermia was found at sperm analysis. The dexamethasone dose was increased to a higher dose (1.5 mg/24 h) for replacement therapy. After 6 months, the testicular sizes were reduced but high glucocorticoid doses induced side effects. Surgical intervention was considered due to the persistence of tumor despite overtreatment. At the operation bilateral, a truffle-like noduler tumor was enucleated with testic- ular tissue sparing. Histopathalogic examination of the formalin-fixed tissue showed large, polygonal, eosinophilic cells with T. Tiryaki (&) Æ H. Atayurt Pediatric Surgery Clinic, Social Security Institution Children’s Hospital, Ankara, Turkey E-mail: httiryaki@ hotmail.com Z. Aycan Department of Pediatric Endocrinology, Social Security Institution Dıskapı Hospital, Ankara, Turkey S. Hu¨cu¨menog˘lu Pathology Clinic, Social Security Institution Dıskapı Hospital, Ankara, Turkey T. Tiryaki Atatu¨rk Bulvarı Erciyes, Han No 109/14, Kızılay, Ankara, Turkey Pediatr Surg Int (2005) 21: 853–855 DOI 10.1007/s00383-005-1547-x