Seminoma in a 46,XX true hermaphrodite with positive H-Y antigen. A case report

This case study represents the first report of a 46,XX phenotypic male true hermaphrodite with a pure seminoma. Serologic testing of tissue demonstrated the presence of the H-Y antigen. This finding supports the theory that the H-Y gene is essential for primary sex determination and raises the possibility that it may also be the specific factor responsible for malignant degeneration of dysgenetic gonads.