Publication:

Incidental gonadoblastoma in swyer syndrome: a case report with brief review of literature

Swyer syndrome is a disorder of sexual differentiation with an incidence of 1 in 80,000 population. Dysgenetic gonads have a propensity for malignant transformation particularly in the presence of Y chromosome and hence need prophylactic removal. We report a case of an adolescent girl who presented with primary amenorrhea who was identified as a case of 46 XY dysgenesis after karyotype studies. Extirpation of gonads were done laparoscopically and on histopathological assessment gonadoblastoma was detected. This case report aims to reiterate the importance of gonadectomy in patients with swyer syndrome as tumors could arise even in the absence of frank adnexal masses.