Testis and epididymis
Intersex syndromes
Gonadal dysgenesis - pure

Author: Turki Al-Hussain, M.D. (see Authors page)

Revised: 6 April 2015, last major update September 2012

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PubMed Search: Gonadal dysgenesis [title] pure

Cite this page: Gonadal dysgenesis - pure. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/testisgonadaldyspure.html. Accessed December 11th, 2017.
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

Images hosted on other servers:

Woman with dysgerminoma and streak gonad

Microscopic (histologic) images

Dysgerminoma

Streak gonad