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Abnormal streak gonads in 46,XY complete gonadal dysgenesis
1Division of Reproductive Endocrinology and Infertility, Department of Obstetrics, Gynecology, and Reproductive Sciences, Yale University School of Medicine, New Haven, Connecticut 06511, USA.
Objective:
To describe the surgical findings in two adolescents with 46,XY complete gonadal dysgenesis.
Design:
Report of two cases.
Setting:
University teaching hospital.
Patient(S):
Two adolescents with 46,XY complete gonadal dysgenesis who underwent laparoscopic bilateral gonadectomy.
Intervention(S):
Laparoscopic bilateral gonadectomy.
Main Outcome Measure(S):
Removal of dysgenetic gonads.
Result(S):
One benign fibrothecoma and one gonadoblastoma were identified upon pathologic evaluation of the streak gonads.
Conclusion(S):
Individuals with 46,XY complete gonadal dysgenesis are at risk for malignant transformation of the dysgenetic gonads. Because the risk of malignancy is approximately 30%, prompt gonadectomy should be performed after diagnosis of 46,XY complete gonadal dysgenesis.
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