In humans, gonadoblastoma arises in undifferentiated gondal tissue within dysgenetic gonads of intersex patients or phenotypic females carrying at least a part of Y chromosome that includes the GBY locus with an estimated risk of up to 30% [29,60], and human TSPY is at the moment the most likely candidate gene for GBY. This evidence concerns the gene TSPY1 and gonadoblastoma.