LAUSR

OBJECTIVE The aim of this study was to assess the prevalence of ovarian and paraovarian adrenal rest tumors (ARTs) in gonadectomy materials of a subgroup of congenital adrenal hyperplasia (CAH) patients. METHODS A total of 20 historical cases with clinical/molecular diagnosis of classical CAH were included in the study. All patients had 46,XX karyotype and underwent gonadectomy because of being raised as male. RESULTS Median age at diagnosis of CAH was 5.7 years and markedly delayed. All patients revealed severe virilization. Bone age was significantly advanced, and bone age/chronological age ratio was increased with a median ratio of 1.8. Median age at the time of gonadectomy was 9.2 years. Ovarian and paraovarian ARTs were detected on pathological evaluation of gonadectomy materials in 4 patients (20%) (2 with simple virilizing 21-hydroxylase, 2 with 11-beta-hydroxylase deficiency) with previously normal pelvic imaging. In three cases with ARTs, paraovarian area was composed of medium-size polygonal cells, with round or oval monomorphic nuclei and abundant granular eosinophilic cytoplasm which is characteristic of adrenocortical tissue. The fourth case had bilateral ovarian "steroid cell tumors, not otherwise specified" and the tumor was accepted as benign. Except for the ARTs, heterotopic prostate and bilateral paratubal epididymis tissue were detected in a patient. CONCLUSIONS Ovarian and paraovarian ARTs might be more common than previously described, especially among patients with excessive and prolonged ACTH exposure. These tumors could be detected histopathologically even if not detected by classical imaging methods.