Bilateral gonadoblastoma in a 9-month-old infant with 46,XY gonadal dysgenesis
A 9-month-old infant with hypertrophic clitoris and separated urethral and vaginal opening was diagnosed as having 46,XY incomplete “pure gonadal dysgenesis”. Examination of both gonads revealed gonadoblastoma and bilateral salpingo-gonadectomy and partial hysterectomy were performed. Patient is raised as a girl, and clitoroplasty will be done in next several months. The fact that gonadal neoplasia in our patient was found already at the age of 9 months, confirms the need for gonadectomy at the time of diagnosis in the patients with 46,XY gonadal dysgenesis.
KeywordsPure gonadal dysgenesis gonadoblastoma infancy intersex
Unable to display preview. Download preview PDF.
- 1.Manuel M., Katayama K.P., Jones H.W. The age of occurrence of gonadal tumors in intersex patients with a Y chromosome. Am. J. Obstet. Gynecol. 124: 193, 1976.Google Scholar
- 4.Damjanov I., Klauber G. Microscopic gonadoblastoma in dysgenetic gonad of an infant. An ultrastructural study. Uropathology 75: 605, 1980.Google Scholar