title: Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia: prevalence and sonographic, hormonal, and seminal characteristics.
abstract: OBJECTIVE
  Testicular adrenal rest tumors have been described in patients with congenital adrenal hyperplasia (CAH). The aim of this work was to (1) evaluate the prevalence of testicular adrenal rest tumors in patients with CAH; (2) study the hormonal profile; (3) define the sonographic features; (4) assess the seminal profile; and (5) initiate a longitudinal study on the possible role of corticotropin (ACTH) plasma levels in the induction and persistence of testicular adrenal rest tumors.
  
  
  METHODS
  Eighteen patients affected by CAH, aged 21 to 41 years, were studied. These were all patients referred to our endocrinology unit for the first time to undergo a clinical evaluation. All of the patients were taking long-term cortisone acetate and fludrocortisone replacement therapy. The study included (1) a physical examination, (2) testis sonography, (3) a hormonal profile, (4) semen analysis.
  
  
  RESULTS
  Sonography showed testicular adrenal rest tumors in 11 patients (61.1%); of these, 9 cases (50.0%) were bilateral, and 2 (11.1%) were unilateral. The diameter ranged from 4 to 38 mm. In 9 patients, the lesions were hypoechoic, whereas in 2, they were hyperechoic. High plasma ACTH levels were detected in all of the patients with tumors despite long-term therapy. Semen analysis found 2 cases of azoospermia and 6 cases of oligoasthenoteratozoospermia; the 3 remaining patients were normospermic. The preliminary longitudinal study has shown 3 patients with a disappearance or reduction of the tumors after 6 months of modified treatment.
  
  
  CONCLUSIONS
  This study confirms the high prevalence of testicular adrenal rest tumors in patients with CAH and the major role played in its pathogenesis by high plasma ACTH levels.
authors.affiliation: Department of Medical and Molecular Medicine, Unit of Andrology, Sant’Andrea Hospital, University of Rome Sapienza, Rome, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Delfino
authors.forename: Jlenia
authors.initials: J
authors.lastname: Elia
authors.forename: Norina
authors.initials: N
authors.lastname: Imbrogno
authors.forename: Nicola
authors.initials: N
authors.lastname: Argese
authors.forename: Rossella
authors.initials: R
authors.lastname: Mazzilli
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Toscano
authors.forename: Fernando
authors.initials: F
authors.lastname: Mazzilli
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000150:complications*; D000314:Adrenal Rest Tumor / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D014408:Biomarkers, Tumor / Q000097:blood; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D015995:Prevalence; D055101:Semen Analysis; D013736:Testicular Neoplasms / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000209:etiology*; D018615:Ultrasonography, Doppler, Color
chemical_list: D014408:Biomarkers, Tumor; D000324:Adrenocorticotropic Hormone