CASE REPORTS
JOURNAL ARTICLE
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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.

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