46,XY pure gonadal dysgenesis: clinical presentations and management of the tumor risk.

@article{Capito201146XYPG,
  title={46,XY pure gonadal dysgenesis: clinical presentations and management of the tumor risk.},
  author={Carmen Capito and Marc-David Leclair and Alexis Arnaud and Albert David and Sabine Baron and Nad{\`e}ge Corradini and Yves H{\'e}loury},
  journal={Journal of pediatric urology},
  year={2011},
  volume={7 1},
  pages={
          72-5
        }
}
PATIENTS Eleven patients with 46,XY PGD were divided into two groups. Six symptomatic girls (group 1) were referred for amenorrhea (n = 3), gonadal tumor (n = 2) or campomelic dysplasia (n = 1). Five asymptomatic screened patients (group 2) were diagnosed as 46,XY PGD after familial investigation of the two probands with gonadal tumor. Bilateral gonadectomy was performed in all patients. RESULTS In group 1, pathologic examination revealed an association of dysgerminoma with gonadoblastoma (n… CONTINUE READING

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