GRANULOSA-CELL TUMOR CAUSING PRECOCIOUS P SEUDOPUBERTY

Three prepubertal girls with granulosa cell tumors presented with prec ocious pseudopuberty and a palpable pelvic tumor. Elevated estrogen le vels and failure of gonadotropin responses after gonadotropin releasin g hormone stimulation were the classical findings in endocrinological testing. Combined with abdominal sonography to show the tumors with so lid and cystic components the diagnosis of granulosa cell tumor can be made with high probability before surgery. In two patients tumors wer e totally removed by salpingo-ovarectomy. In one of the patients the t umor ruptured during surgery. One to eleven years after surgery all th ree girls are alive without any evidence of disease. Already in the se cond postoperative week estrogen levels decreased and interaction betw een the hypothalamic-pituitary system and the remaining ovary was rees tablished. Clinical signs of precocious pseudopuberty together with el evated estrogen levels and failure of gonadotropin responses, palpable tumor and the typical ultrasound findings are usually so characterist ic that preoperative diagnosis can be made.