Ovotesticular disorder of sex development with a prostatic gland and review of literature

Andrologia. 2009 Dec;41(6):387-91. doi: 10.1111/j.1439-0272.2009.00945.x.

Abstract

Ovotesticular disorder of sex development (OTDSD) is a rare condition and defined as the presence of ovarian and testicular tissue in the same individual. Most of patients with OTDSD have female internal genital organs. In this report, we present a case in which, we demonstrated prostate tissue using endoscopic and radiologic methods in a 46-XX, sex determining region of the Y chromosome negative male phenotypic patient, with no female internal genitalia. Existence of prostate in an XX male without SRY is rarely seen and reveals a complete male phenotype. This finding is critical to figure out what happens in embryonal period.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Cystoscopy
  • Gynecomastia / etiology
  • Humans
  • Karyotyping
  • Magnetic Resonance Imaging
  • Male
  • Ovotesticular Disorders of Sex Development / complications
  • Ovotesticular Disorders of Sex Development / diagnosis*
  • Ovotesticular Disorders of Sex Development / genetics
  • Phenotype
  • Prostate / pathology*
  • Scrotum / diagnostic imaging
  • Sex-Determining Region Y Protein
  • Ultrasonography

Substances

  • Sex-Determining Region Y Protein