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Macroorchidism: Consequence of untreated congenital adrenal hyperplasia

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Abstract

Testicular adrenal rest tumors (TART) are consequences of delayed diagnosis and/or undertreatment in patients with congenital adrenal hyperplasia (CAH). We describe a case of CAH with TART who presented with bilateral macroorchidism. He was managed with glucocorticoids which led to decrease in testicular size without restoration of spermatogenesis.

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References

  1. Claahsen-van der Grinten HL, Otten BJ, Sweep FC, Span PN, Ross HA, Meuleman EJ, et al. Testicular tumours in patients with congenital adrenal hyperplasia due to 2lhydroxylase deficiency show functional features of adrenocortical tissue. J Clin Endocrinol Metab. 2007; 92:3674–3680.

    Article  PubMed  CAS  Google Scholar 

  2. Avila NA, Premkumar A, Merke DP. Testicular adrenal rest tissue in congenital adrenal hyperplasia: comparison of MR imaging and sonographic findings. Am J Roentgenol. 1999;172:1003–1006.

    CAS  Google Scholar 

  3. Claahsen-van der Grinten HL, Sweep FCGJ, Blickman JG, Hermus A RMM, Otten BJ. Prevalence of testicular adrenal rest tumors in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Eur J Endocrinol. 2007;157:339–344.

    Article  PubMed  CAS  Google Scholar 

  4. Souverijns G, Peene P, Keuleers H, Vanbockrijck M. Ectopic localization of adrenal cortex. Eur Radiol. 2000;10:1165–1168.

    Article  PubMed  CAS  Google Scholar 

  5. Reisch N, Scherr M, Flade L, Bidlingmaier M, Schwarz HP, Müller-Lisse U, et al. Total adrenal volume but not testicular adrenal volume is associated with normal control in patients with 21-hydroxylase deficiency. J Clin Endocrinol Metabol. 2010;95:2065–2072.

    Article  CAS  Google Scholar 

  6. Claahsen-van der Grinten HL, Otten BJ, Stickelbroeck MML, Sweep FCGJ, Hermus ARMM. Testicular adrenal rest tumours in congenital adrenal hyperplasia. Best Pract Res Clin Endocrinol Metabol. 2009;23:209–220.

    Article  CAS  Google Scholar 

  7. Claahsen-van der Grinten HL, Otten BJ, Hermus AR, Sweep FC, Hulsbergen-van de Kaa CA. Testicular adrenal rest tumours in patients with congenital adrenal hyperplasia can cause severe testicular damage. Fertil Steril. 2008;89:597–601.

    Article  PubMed  Google Scholar 

  8. Bercovici JP, Fiet J, Gibault L, Volant A, Abalain JH, Floch HH, et al. Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo and in vitro studies). J Steroid Biochem Mol Biols. 2005;93:67–72.

    Article  CAS  Google Scholar 

  9. White CP, Carter JN. Adrenal and testicular tumors and azoospermia in congenital adrenal hyperplasia. Aust New Zeal J Med. 1993;29:410.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Endocrinology, PGIMER, Chandigarh, 160 012, India

    Ashu Rastogi, Rama Walia & Anil Bhansali

  2. Department of Pathology, PGIMER, Chandigarh, 160 012, India

    Uma Nahar Saikia

Authors
  1. Ashu Rastogi
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  2. Rama Walia
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  3. Uma Nahar Saikia
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  4. Anil Bhansali
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Correspondence to Anil Bhansali.

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Rastogi, A., Walia, R., Saikia, U.N. et al. Macroorchidism: Consequence of untreated congenital adrenal hyperplasia. Indian Pediatr 49, 577–578 (2012). https://doi.org/10.1007/s13312-012-0096-3

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  • DOI: https://doi.org/10.1007/s13312-012-0096-3

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