금번 저자들은 외견적인 표현형이 정상여성이고 양측에 삭상성선이 있으며, 좌측에 미분화 세포종을 동반한 생식아세포종을 가진 46, XY 순수생식선이형성증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다

A case of 46, XY pure gonadal dysgenesis who showed phenotypic female with bilateral streak gonads complicated by an unilateral gonadoblastoma overgrown with dysgerminoma and an infantile uterus has been presented The neoplastic potential of dysgenetic gonads in an individual with a Y-chromosome has been reviewed with literatures and therefore the need for complete bilateral gonadectomy in such a case has been stressed upon