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Ovary - nontumor

Gonadal dysgenesis

Female pseudohermaphroditism-non adrenal


Reviewer: Mohiedean Ghofrani, M.D, (see Reviewers page)
Revised: 28 December 2011, last major update December 2011
Copyright: (c) 2002-2012, PathologyOutlines.com, Inc.

General
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● The rarer form of female pseudohermaphroditism in which a female genotype (46,XX) and female internal phenotype (two ovaries) is associated with variable degrees of virilization due to etiologies other than congenital adrenal hyperplasia, including gestational hyperandrogenism (maternal exposure to progestins or androgens)
● 46XX, Barr bodies are chromatin positive
● Ovary with female ducts and variable virilized external genitalia

Terminology
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● In 2006, the International Consensus Conference on Intersex recommended using the designation "46,XX DSD" to replace the potentially pejorative and confusing term female "pseudohermaphroditism" (Pediatrics 2006;118:e488)

Laboratory
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● 17-ketosteroids and estrogen levels are normal

Epidemiology
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● Gestational hyperandrogenism is a rarer cause of 46,XX DSD

Etiology
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● May be due to exposure to maternal androgen (maternal luteoma, theca-lutein cysts, placental aromatase enzyme deficiency) or synthetic progestational agents

Clinical Features
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● Diagnosis may be suggested by history of exposure to exogenous progestin or androgen or maternal virilization

Case reports
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● With luteoma of pregnancy (Hum Reprod 2002;17:821)

Additional references
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eMedicine

End of Ovary - nontumor > Gonadal dysgenesis > Female pseudohermaphroditism-non adrenal


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