Bilateral ovarian gonadoblastoma with coexisting dysgerminoma in a girl with 46, XX karyotype

Gonadoblastoma is a rare gonadal tumor with tumor cells arranged in nests surrounded by ovarian stroma containing Leydig or lutein-type cells. In 50% of the cases, there is an overgrowth of germ cells with progression to dysgerminoma. A case of gonadoblastoma with coexisting dysgerminoma developing in both ovaries of a 20-year-old girl who had increased abdominal girth is presented here. A pelvic mass measuring 20 × 14 cm was detected by pelvic ultrasonography. Bilateral salphingo-oophorectomy was performed. The histopathological report revealed bilateral gonadoblastoma with coexisting dysgerminoma. After surgery the patient received radiation and chemotherapy (BEP: bleomycin, etoposide, cisplatin) and was started on hormone replacement therapy. Five years and eight months after treatment, the patient is well and free of recurrence.