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.
Division of Reproductive Endocrinology and Infertility, Department of Obstetrics, Gynecology, and Reproductive Sciences, Yale University School of Medicine, New Haven, Connecticut 06511, USA.
Leo F
LF
Doherty
Beth W
BW
Rackow
D000293:Adolescent; D005260:Female; D006061:Gonadal Dysgenesis, 46,XY / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D018238:Gonadoblastoma / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D006066:Gonads / Q000002:abnormalities* / Q000473:pathology; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology