Swyer Syndrome with Gonadoblastoma: A Case Report

These patients have female external phenotype and usually presented with primary amenorrhea.Detailed history, clinical, radiological and hormonal evaluation, karyotyping and histopathologyare needed for diagnosis. Due to the presence of Y chromosomes these patients have streak gonadswith increased risk of development of gonadoblastoma. Early diagnosis of Swyer syndromefollowed by prophylactic gonadectomy is very important. Case presentation: A 19-year-old female presented with the complaints of primary amenorrhea.The clinical, radiological, hormonal assessment and karyotyping test made the diagnosis of Swyersyndrome. The histopathological examination revealed left sided streak gonad and right sidedgonadoblastoma.Conclusion: Swyer syndrome is a rare form of disorders of sexual development. As there is highchance of gonadal malignancy, these patients should be counseled for preventive bilateral salpingooophorectomy