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

✍ Scribed by Aleksander Talerman; Marion S. Verp; Elizabeth Senekjian; Theresa Gilewski; Nicholas Vogelzang

• Book ID: 102673486
• Publisher: John Wiley and Sons
• Year: 1990
• Tongue: English
• Weight: 747 KB
• Volume: 66
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/1097-0142(19901215)66:12<2668::aid-cncr2820661234>3.0.co;2-5

✦ Synopsis

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.