Testis & epididymis
Gonadal dysgenesis-pure

Topic Completed: 1 September 2012

Minor changes: 8 September 2020

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PubMed Search: Gonadal dysgenesis [title] pure

Turki Al-Hussain, M.D.
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Cite this page: Al-Hussain T. Gonadal dysgenesis-pure. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testisgonadaldyspure.html. Accessed September 21st, 2020.
Definition / general
  • Also called Swyer syndrome
  • Female phenotype and female genital appearance, normal Müllerian structures
  • Bilateral streak gonads, so no hormonal or reproductive potential
  • 46 XY but no testicular tissue; no evidence of Turner syndrome
  • Early bilateral gonadectomy is recommended due to high incidence of gonadoblastoma and dysgerminoma (J Pediatr Urol 2011;7:72)
Case reports
Gross images

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Woman with dysgerminoma and streak gonad

Microscopic (histologic) images

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Streak gonad

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