Gonadoblastoma and chroiocarcinoma in dysgenic gonads: report of a case

Dysgenetic gonads are well known to be associated with gonadoblastoma and various germ cell tumors, but very rarely with choriocarcinoma. We describe a 16-year-old, phenotypic female patient with a 46XY karyotype who developed gonadoblastoma on the right gonad and choriocarcinoma on the left. There was no co-existence of the two tumor cell types in the same gonad. This case emphasizes the importance of bilateral gonadectomy for "46XY female" patients, as soon as the cytogenetic evidence is known.