Gonadal dysgerminoma-seminoma associated with severe hypercalcemia

A case is reported, of a 25-year-old phenotypic female, karyotypic male patient with metastatic gonadal dysgerminoma-seminoma and severe hypercalcemic paraneoplastic syndrome. Patient manifested normal female external genitalia, vagina, uterus, and fallopian tubes but with 46 XY karyotype. Adnexal gonads were calcified. External beam radiation therapy resolved the hypercalcemic state. Exploratory laparotomy after irradiation revealed gonadoblastoma originating from dysgenetic gonads.