Abnormal streak gonads in 46,XY complete gonadal dysgenesis.

Objective: To describe the surgical findings in two adolescents with 46,XY complete gonadal dysgenesis.

Methods: Report of two cases. Methods: University teaching hospital. Methods: Two adolescents with 46,XY complete gonadal dysgenesis who underwent laparoscopic bilateral gonadectomy. Methods: Laparoscopic bilateral gonadectomy. Methods: Removal of dysgenetic gonads.

Results: One benign fibrothecoma and one gonadoblastoma were identified upon pathologic evaluation of the streak gonads.

Conclusions: 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.