A patient with Swyer sydrome presenting with gonadoblastoma and dysgerminoma

Abstract

Swyer syndrome or 46 XY gonadal dysgenesis is characterized by a female phenotype with streak gonads and normal female external genitalia and complicated by the frequent occurrence of germ cell tumors. Gonadoblastoma and dysgermi¬noma are the most frequently reported malignancies. A 15 year-old female with clitoris hypertrophy and pelvic mass was found to have a gonadoblastoma in the left ovary and dysgerminoma in the right ovary. Her karyotype was 46 XY. These two synchronous tumours, made this case rare and interesting.