Clinical, endocrine and ultrastructural study of XY gonadal dysgenesis. A case report.

About 120 cases of XY gonadal dysgenesis have been reported on. We treated such a patient with bilateral gonadectomy. The gonadal tissue's capacity to respond to hormonal trophic stimulation was assessed. When the gonads were examined ultrastructurally, structures with the morphologic characteristics of stromal ovarian cells, Sertoli's cells and Leydig's cells were found. Because of the potential malignancy of the XY gonads, bilateral gonadectomy and hormonal substitution therapy are recommended for these patients. We prefer to use combined hormone replacement with sequential estrogen and progesterone rather than sequential unopposed estrogen because of the small but increased risk of endometrial hyperplasia and carcinoma after long-standing sequential therapy.