Ovarian dysgerminoma with contralateral gonadoblastoma in a 46XY phenotypic female: Swyer syndrome

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1996

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Abstract

A 15-year-old girl was admitted to the pediatric surgery department with primary amenorrhea and right inguinal swelling of one year duration. On physical examination, the patient presented with a female phenotype, but no breast development; somewhat hirsute with hypertrophic clitoris. Ultrasound examination disclosed a palpable mass originating from the right ovary. At operation, a right salpingo-ooferectomy was performed, and a biopsy specimen was obtained from the left ovary which did not appear normal. Histopathologic examination revealed a right ovarian disgerminoma and a left gonadoblastoma. One month after the first operation, a left gonadectomy was performed with accompanying cliteroplasty. An original case of Swyer syndrome is reported in a patient with female phenotype, dysgerminoma of the right and gonadoblastoma of the the left ovaries.

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