Abnormal streak gonads in 46,XY complete gonadal dysgenesis.

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.