Abstract
BackgroundDysgerminoma is the most common malignant germ cell tumor of the ovary. This malignancy can be associated with pure gonadal dysgenesis or Swyer syndrome, mixed gonadal dysgenesis and partial gonadal dysgenesis.Case presentationDysgerminoma developed in 3 phenotypic female patients with 46 XY pure gonadal dysgenesis. All patients presented first with abdominopelvic mass. Laparatomy was done. 46 XY karyotype was made by lymphocyte culture. Then these patients underwent gonadectomy that histopathology results were streak ovaries without evidence for malignancy. Two patients received postoperative adjuvant therapy.ConclusionIn Patients with Swyer syndrome the risk of dysgerminoma is high and gonadectomy is recommended. Also 5% of dysgerminomas are discovered in phenotypic female and 46 XY karyotype, thus in adolescent with dysgerminoimas and amenorrhea, karyotype should be done.
Highlights
Dysgerminoma is the most common malignant germ cell tumor of the ovary
In Patients with Swyer syndrome the risk of dysgerminoma is high and gonadectomy is recommended
5% of dysgerminomas are discovered in phenotypic female and 46 XY karyotype, in adolescent with dysgerminoimas and amenorrhea, karyotype should be done
Summary
Bilateral dysgenesis of the testes (Swyer syndrome) affected individuals has an XY karyotype but normal (infantile) female external and internal genitalia. In these patients the risk of dysgerminoma is high and gonadectomy was recommended. In 5% of cases of dysgerminoma, XY karyotype is seen. In adolescents patients with dysgerminoma with primary amenorrhea and secondary amenorrhea, Karyotype should be done. Menstrual function in patients with 46 XY karyotype may be associated with estrogen secretion of tumoral lesion and investigation of gonads is recommended
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