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Dysgerminoma … Gonadoblastoma and Familial 46XY Pure Gonadal Dysgenesis: Case Report and Review of the Genetics and Pathophysiology of Gonadal Dysgenesis and H‐Y Antigen

Summary: 46XY gonadal dysgenesis is a rare condition of intersexuality first described in detail by Swyer in 1955. It is characterized by a phenotypic female who is tall, eunuchoid with normal female external genitalia, hypoplastic uterus and Fallopian tubes, streak ovaries and primary amenorrhoea. The patients exhibit none of the associated congenital defects such as webbing of the neck and genu valgum seen in Turner's syndrome. Although rare, this clinical entity is important to recognize because of the high incidence of gonadoblastomas and subsequent development of dysgerminoma. The condition may be familial and therefore siblings need to be screened. To the geneticists and scientists researching in the area of intersexuality, hermaphroditism and sexual determination, it has helped to unveil the mechanisms of gonadal and genital sexual development.