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True hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, 46, XX/46, XY karyotype, and a successful pregnancy
Author(s) -
Talerman Aleksander,
Verp Marion S.,
Senekjian Elizabeth,
Gilewski Theresa,
Vogelzang Nicholas
Publication year - 1990
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19901215)66:12<2668::aid-cncr2820661234>3.0.co;2-5
Subject(s) - gonadoblastoma , ovotestis , dysgerminoma , gonad , true hermaphroditism , karyotype , gonadal dysgenesis , y chromosome , hermaphrodite , gynecology , medicine , pathology , ovary , biology , anatomy , chromosome , genetics , ecology , gene
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.