A combined germ cell--gonadal stromal--epithelial tumor of the ovary

This report describes a previously unreported type of ovarian tumor containing three distinct components: germ cells, gonadal stromal cells, and epithelial cells. The germ cells were mainly of the types seen in various stages of oogenesis. The gonadal stromal component had two lines of differentiation. Cells containing Charcot-Böttcher filaments were identified as Sertoli cells and granulosa cells were characterized by a microfollicular arrangement. The epithelial component resembled the cells present on the ovarian surface and lacked specific differentiation toward any of the common ovarian (müllerian) epithelial cell lines. The tumor was discovered as an abdominal mass in a newborn girl with a 46XX karyotype. Subsequently, the child has developed normally and is well 7 years after surgery.