The presence of testicular tumors in male patients with CAH resulting from 21-hydroxylase deficiency was first described in 1940 by Wilkins et al. (15). Since then, the reported prevalence has varied between 0 and 94% (2,3,5,6,9). The reported prevalence is strongly dependent on patient selection (prepubertal, adolescent, or adult), and on the method of tumor detection (physical examination or imaging techniques). Urban et al. (3) found no testicular tumors by physical examination in 30 adult patients. Using ultrasonography, adrenal rest tumors were reported in 2 of 14 adult patients (2), 9 of 18 adult patients (5), and 16 of 17 postpubertal patients (6). Tumors have also been reported in prepuberty: of the 8 patients with testicular tumors described by Avila et al. in a population of 38 patients, 7 were younger than 16 yr old (9). Shanklin et al. reviewed the autopsy material of patients with CAH and noted testicular nodules in 3 of 7 patients younger than 8 wk old and in all 14 patients older than 14 mo (16).
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