A case of bilateral gonadoblastoma in streak gonads

A report of a patient with bilateral gonadoblastomas who sought consultation for primary amenorrhea and infertility is presented. Thelarche and adrenarche occurred spontaneously at age 13. Clitoromegaly and a hypoplastic uterus were noted on physical examination. Flat plate X-ray of the abdomen demonstrated radiopaque material in both adnexa. Urinary 17-ketosteroid levels were normal, but the serum testosterone concentration was elevated. Serum gonadotropin values were markedly elevated, and total serum estrogens levels were very low. The karyotype was XY. The gonads were extirpated, and typical histology is demonstrated. A postulate was made to explain the clinical findings.