Granulosa and theca cell tumors in children: a report of 17 cases and literature review.

ABSTRACT

The files of the Emil Novak Ovarian Tumor Registry (ENOTR) were searched for granulosa and theca cell tumors in children aged 12 years and less. In addition, an extensive literature search was done for English publications on children with these tumors aged 10 years or less. Of the 17 children from the ENOTR, 5 had adult-type granulosa cell tumors, 6 had juvenile granulosa cell tumors, and 1 had a luteinized granulosa cell tumor. In addition, there were three cases with gonadal stomal tumors, one theca cell tumor, and one granulosa-theca cell tumor. Precocious pseudopuberty was present in 70 percent of the children, abdominal pain in 24 percent, and ascites in 18 percent. The literature review revealed a tumor-related mortality rate of 9 percent (based on 163 cases with granulosa cell tumors, including the juvenile type). Some of these tumors are large with acute pain, but nevertheless, the prognosis is good, particularly in cases with precocious puberty. Treatment can be conservative.