Current knowledge indicates that patients with DSDs are at an increased risk for the development of malignancy such as seminoma, gonadoblastoma, and dysgerminoma.[15] The risk for tumor development depends on many parameters, including gonadal morphology, germ cell location, presence of germ cells, genital morphology, patient age, and presence of TSPY.[4, 16] Furthermore, the risk for GCT development varies among different DSDs. This evidence concerns the gene TSPY1 and gonadoblastoma.