A Case of Dysgerminoma Associated with 46, XY Pure Gonadal Dysgenesis / 대한부인종양콜포스코피학회잡지

Dysgerminoma developed in a 21-year-old phenotypic female patient with 46,XY pure gonadal dysgenesis, Swyer syndrome. This patient presented with pelvic mass associated with abdominal pain and primay amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and left streak gonad. A 46,XY karyotype was made by lymphocyte culture. The patient was counseled to undergo operation, chemotherapy and hormon therapy. She underwent bilateral gonadectomy with total hysterectomy, partial omentectomy and multiple pelvic wall random biopsy. Histological examination revealed dysgenetic gonads with dysgerminoma. After surgery, the patient received chemotherapy and also was started on hormone replacement therapy. She is currently alive with no evidence of disease after 19 months from surgery.

Gonadoblastoma and dysgerminoma associated with 46,XY pure gonadal dysgenesis--a case report

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.