Abstract
Women who have a Difference of Sex Development and Y chromosomal material in their (gonadal) karyotype are at increased risk for developing a malignant gonadal germ cell tumor (GCT). These GCT can be either seminomas (dysgerminomas) or non-seminomas and are always preceded by an in situ neoplastic lesion: germ cell neoplasia in situ in a (dysgenetic) testicular context or gonadoblastoma in a context of undifferentiated gonadal tissue. Therefore, prophylactic gonadectomy in childhood has long been a standard procedure.
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