Pseudoprecocity Due to Lesions of the Ovary

Published on 22/03/2015 by admin

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Last modified 22/03/2015

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Chapter 581 Pseudoprecocity Due to Lesions of the Ovary

Ovarian tumors are rare in pediatrics, occurring at a rate of less than 3/100,000. Most ovarian masses are benign, but 10-30% may be malignant. Ovarian malignancies, the most common genital neoplasms in adolescence, account for only 1% of childhood cancers. More than 60% are germ cell tumors, most of which are dysgerminomas that can secrete tumor markers and hormones (Chapter 497). Five to 10% of them occur in phenotypic females with abnormal gonads associated with the presence of a Y chromosome. Next most common are epithelial cell tumors (20%), and nearly 10% are sex cord/stromal tumors (granulosa, Sertoli cell, and mesenchymal tumors). Multiple tumor markers can be seen in ovarian tumors, including α-fetoprotein, human chorionic gonadotropin (hCG), carcinoembryonic antigen, oncoproteins, p105, p53, KRAS mutations, cyclin D1, epidermal growth factor–related proteins and receptors, cathepsin B, and others. Variable levels of inhibin-activin subunit gene expression have been detected in ovarian tumors.

Functioning lesions of the ovary consist of benign cysts or malignant tumors. The majority synthesize estrogens; a few synthesize androgens. The most common estrogen producing ovarian tumor causing precocious puberty is the granulosa cell tumor. Other tumors that can cause precocious puberty are thecomas, luteomas, mixed types, theca-leutein and follicular cysts, and ovarian tumors (i.e., teratoma, choriocarcinoma, and dysgerminoma).

Estrogenic Lesions of the Ovary

These lesions cause isosexual precocious sexual development but account for only a small percentage of all cases of precocity. Benign ovarian follicular cysts are the most common tumors associated with isosexual precocious puberty in girls; they may rarely be gonadotropin dependent.