Gonadoblastoma and dysgerminoma associated with 46,XY pure gonadal dysgenesis--a case report.
10.3346/jkms.1993.8.5.380
- Author:
Sei Kwang KIM
1
;
In Sook SOHN
;
Jae Wook KIM
;
Chan Ho SONG
;
Chan Il PARK
;
Moo Sang LEE
;
Gwi Won KIM
;
Kyu Rae KIM
Author Information
1. Department of Obstetrics and Gynecology, Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Reports
- Keywords:
Gonadoblastoma;
Dysgerminoma;
46;
XY Pure Gonadal Dysgenesis;
Gonadectomy
- MeSH:
Adult;
Dysgerminoma/*etiology/pathology/therapy;
Female;
Gonadal Dysgenesis, 46,XY/*complications;
Gonadoblastoma/*etiology/pathology/therapy;
Humans;
Ovarian Neoplasms/*etiology/pathology/therapy
- From:Journal of Korean Medical Science
1993;8(5):380-384
- CountryRepublic of Korea
- Language:English
-
Abstract:
Gonadoblastoma and dysgerminoma developed in a 24-year-old phenotypic female patient with 46,XY pure gonadal dysgenesis. This patient presented with primary amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and bilateral streak gonads. A 46,XY karyotype was made by lymphocyte culture. The patient was counseled to undergo a prophylactic bilateral gonadectomy, but she refused. Three years and three months after the initial diagnosis she felt a growing pelvic mass. Bilateral gonadectomy and total hysterectomy were performed. Histological examination revealed gonadoblastoma and dysgerminoma on both gonads. After surgery the patient received radiation therapy and also was started on hormone replacement therapy. Two years and two months after treatment by surgery the patient is well and free of recurrence.
