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.

Juvenile Granulosa Cell Tumor

In childhood, the most common neoplasm of the ovary with estrogenic manifestations is the granulosa cell tumor, although it makes up only 1-10% of all ovarian tumors. These tumors have distinctive histologic features that differ from those encountered in older women (adult granulosa cell tumor). The cells have high mitotic activity, follicles are often irregular, Call-Exner bodies are rare, and luteinization is frequent. The tumor may be solid or cystic, or both. It usually is benign. In a few instances, this tumor has been associated with multiple enchondromas (Ollier disease) and, in fewer still, with multiple subcutaneous hemangiomas (Maffucci syndrome).

Clinical Manifestations and Diagnosis

The tumor has been observed in newborns and may manifest with sexual precocity at 2 yr of age or younger; about half these tumors have occurred before 10 yr of age. The mean age at diagnosis is 7.5 yr. The tumors are almost always unilateral. The breasts become enlarged, rounded, and firm and the nipples prominent. The external genitals resemble those of a normal girl at puberty, and the uterus is enlarged. A white vaginal discharge is followed by irregular or cyclic menstruation. Ovulation, however, does not occur. The presenting manifestation may be abdominal pain or swelling. Pubic hair is usually absent unless there is mild virilization.

A mass is readily palpable in the lower portion of the abdomen in most children by the time sexual precocity is evident. The tumor may be small, however, and escape detection even on careful rectal and abdominal examination; the tumors may be detected by ultrasonography, but multidetector CT scans are most sensitive. Most such tumors (90%) are diagnosed at very early stages of malignancy (FIGO [International Federation of Gynecology and Obstetrics] stage I).

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