FEMALE PSEUDOHERMAPHRODITISM DUE TO CONGENITAL ADRENAL-HYPERPLASIA COMPLICATED BY ADENOCARCINOMA OF THE PROSTATE AND CLEAR-CELL CARCINOMA OF THE ENDOMETRIUM

The presence of prostatic glandular tissue in female pseudohermaphrodi tes has previously been documented. However, prostatic neoplasia in th is clinical setting has not been reported, A karyotypic female with co ngenital adrenal hyperplasia due to 21-hydroxylase deficiency develope d a prostatic adenocarcinoma associated with elevated serum prostatic specific antigen levels and osteoblastic skeletal metastases, This dem onstrates that this tissue in pseudohermaphrodites can become malignan t, In addition, the patient subsequently developed clear cell carcinom a of the endometrium, possibly related to radiation therapy for the pr ostatic adenocarcinoma. This demonstrates that female pseudohermaphrod ites may be at risk not only for malignancies seen in genotypic female s but also prostate cancer.