Granulosa cell tumor causing precocious pseudopuberty

Three prepubertal girls with granulosa cell tumors presented with precocious pseudopuberty and a palpable pelvic tumor. Elevated estrogen levels and failure of gonadotropin responses after gonadotropin releasing hormone stimulation were the classical findings in endocrinological testing. Combined with abdominal sonography to show the tumors with solid and cystic components the diagnosis of granulosa cell tumor can be made with high probability before surgery. In two patients tumors were totally removed by salpingo-ovarectomy. In one of the patients the tumor ruptured during surgery. One to eleven years after surgery all three girls are alive without any evidence of disease. Already in the second postoperative week estrogen levels decreased and interaction between the hypothalamic-pituitary system and the remaining ovary was reestablished. Clinical signs of precocious pseudopuberty together with elevated estrogen levels and failure of gonadotropin responses, palpable tumor and the typical ultrasound findings are usually so characteristic that preoperative diagnosis can be made.