True hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, 46, XX/46, XY karyotype, and a successful pregnancy

The first case (to the authors' knowledge) is reported of a true hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, a 46, XX/46,XY karyotype, and a successful pregnancy. the true hermaphroditism was diagnosed during infancy. the patient was subsequently found t...

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Bibliographic Details
Published in:Cancer 1990-12, Vol.66 (12), p.2668-2672
Main Authors: Talerman, Aleksander, Verp, Marion S., Senekjian, Elizabeth, Gilewski, Theresa, Vogelzang, Nicholas
Format: Article
Language:English
Subjects:
Disorders of Sex Development - complications
Disorders of Sex Development - genetics
Disorders of Sex Development - pathology
Dysgerminoma - complications
Dysgerminoma - pathology
Female
Gonads - pathology
Humans
Karyotyping
Neoplasms, Multiple Primary - pathology
Pregnancy
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Summary:The first case (to the authors' knowledge) is reported of a true hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, a 46, XX/46,XY karyotype, and a successful pregnancy. the true hermaphroditism was diagnosed during infancy. the patient was subsequently found to have a gonadoblastoma and a microscopic dysgerminoma in the gonad diagnosed as an ovotestis and excised during infancy. the successful pregnancy occurred when the patient was 29 years old. A year later a large gonadal tumor affecting the remaining gonad was excised. the gonad was found to be an ovotestis, and the tumor was a dysgerminoma arising from a gonadoblastoma. This case further emphasizes the malignant potential of the Y chromosome in patients with abnormal gonads.
ISSN:0008-543X
1097-0142
DOI:10.1002/1097-0142(19901215)66:12<2668::AID-CNCR2820661234>3.0.CO;2-5