title: Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue.
abstract: BACKGROUND
  In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors are frequently found that may interfere with gonadal function.
  
  
  OBJECTIVE
  Our objective was to determine steroid-producing features of testicular adrenal rest tumors.
  
  
  METHODS
  The study is descriptive and took place at a university medical center.
  
  
  METHODS
  Eight adult CAH patients with bilateral testicular adrenal rest tumors were treated with testis-sparing surgery.
  
  
  METHODS
  In all but one patient, spermatic veins were cannulated during surgery and blood samples collected to measure the adrenal-specific steroid 21-deoxycortisol (21DF) and 17-hydroxyprogesterone (17OHP) and androstenedione (A). The same parameters were measured in simultaneously taken peripheral blood. mRNA concentrations of adrenal-specific enzymes CYP11B1 and CYP11B2 and ACTH and angiotensin II (AII) receptors were measured in tumor tissue.
  
  
  METHODS
  Adrenal-specific steroids/enzymes were assessed.
  
  
  RESULTS
  21DF, 17OHP, and A levels were measurable in all spermatic vein samples. The ratio (mean +/- SD) between spermatic vein and simultaneously taken peripheral blood samples was 37.8 +/- 56.3 (21DF), 132.0 +/- 249 (17OHP), and 57.0 +/- 68.2 (A). CYP11B1, CYP11B2, and ACTH and AII receptor mRNAs were detected in all tumors with a strong correlation between ACTH receptor mRNA in tumors and 21DF (r = 0.85; P = 0.015), 17OHP (r = 1; P = 0.01) and A (r = 0.89; P = 0.007) concentrations in peripheral blood.
  
  
  CONCLUSIONS
  Testicular adrenal rest tumors produce adrenal-specific steroids and express adrenal-specific enzymes and ACTH and AII receptors, confirming the strong resemblance with adrenal tissue. Because AII receptors are present in tumor tissue, it can be hypothesized that AII may be an additional factor responsible for testicular adrenal rest tumor growth.
authors.affiliation: Radboud University Nijmegen Medical Centre, Department of Paediatric Endocrinology (833), PO Box 9101, 6500 HB Nijmegen, The Netherlands. H.Claahsen@cukz.umcn.nl
authors.forename: Hedi L
authors.initials: HL
authors.lastname: Claahsen-van der Grinten
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Fred C G J
authors.initials: FC
authors.lastname: Sweep
authors.forename: Paul N
authors.initials: PN
authors.lastname: Span
authors.forename: H Alec
authors.initials: HA
authors.lastname: Ross
authors.forename: Eric J H
authors.initials: EJ
authors.lastname: Meuleman
authors.forename: Ad R M M
authors.initials: AR
authors.lastname: Hermus
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000302:Adrenal Cortex / Q000378:metabolism / Q000503:physiopathology*; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology*; D000314:Adrenal Rest Tumor / Q000209:etiology* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D000328:Adult; D000735:Androstenedione / Q000097:blood; D019405:Cytochrome P-450 CYP11B2 / Q000097:blood / Q000235:genetics / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D012333:RNA, Messenger / Q000032:analysis / Q000378:metabolism; D011945:Receptors, Angiotensin / Q000235:genetics / Q000378:metabolism; D018043:Receptors, Corticotropin / Q000235:genetics / Q000378:metabolism; D013252:Steroid 11-beta-Hydroxylase / Q000097:blood / Q000235:genetics / Q000378:metabolism; D013255:Steroid 21-Hydroxylase / Q000097:blood / Q000235:genetics; D013736:Testicular Neoplasms / Q000209:etiology* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D013737:Testis / Q000098:blood supply
chemical_list: D012333:RNA, Messenger; D011945:Receptors, Angiotensin; D018043:Receptors, Corticotropin; D000735:Androstenedione; D019326:17-alpha-Hydroxyprogesterone; D013255:Steroid 21-Hydroxylase; D019405:Cytochrome P-450 CYP11B2; D013252:Steroid 11-beta-Hydroxylase