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 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.
Talerman
A
A
Department of Pathology, University of Chicago, Illinois.
Verp
M S
MS
Senekjian
E
E
Gilewski
T
T
Vogelzang
N
N
D012734:Disorders of Sex Development / Q000150:complications* / Q000235:genetics / Q000473:pathology; D004407:Dysgerminoma / Q000150:complications* / Q000473:pathology; D005260:Female; D006066:Gonads / Q000473:pathology*; D006801:Humans; D007621:Karyotyping; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D011247:Pregnancy