[An unusual hormone-active avarian tumor in a 3-year-old girl]

A case report on an unusual hormone producing ovarian tumor in a 3 year old girl with sexual precocity is given. The serum values of Estradiol and Progesterone were similar to those of mature women during the luteal phase. The LH values were normal, the 17-Ketosteroids and 17-Hydroxycorticosteroids were near the upper limit of the normal range. At laparotomy a smooth, encapsulated tumor of the left ovary, 12 cm in diameter, was removed by unilateral salpingo-oophorectomy. Hormone analyses of the blood of the ovarian vein as well as of necrotic parts of the tumor showed values of 3450 and 2750 pg/ml Estradiol, and 70 000 and 75 000 pg/ml Progesterone respectively. Histiologically the tumor cells were growing in nests and broad columns, showing bizarre nuclei, abundant pathologic mitoses and patchy calcification. In places the tumor cells were forming follicle-like structures. Both the fat stain and the glycogen stain were positive. Histochemically the tumor showed a strong activity of 3beta-ol-Steroiddehydrogenase. Postoperatively the signs of sexual precocity disappeared soon. The patient is now without complaints for 3 years. The diagnosis of a juvenile granulosa cell tumor (Scully) was given. The endocrine relations between sexual steroids and gonadotropin before and after surgery are discussed.