Juvenile granulosa cell tumors of the ovary in children and adolescents: results from 33 patients registered in a prospective cooperative study.

Clinical and pathological data from 33 prospective registered patients who suffered from juvenile granulosa cell tumors (JGCT) were evaluated according to treatment and outcome. The median age at the time of diagnosis was 7.6 years (range, 6 months to 17.5 years). Fourteen patients showed signs of a pseudo-precocious puberty. In 1 patient premenarcheal bleeding was the only clinical symptom of the disease. A pelvic tumor or an abdominal distention was found in 6 children, revealing signs of an acute abdomen in 3 children. Tumor staging was performed according to the FIGO (International Federation of Gynecology and Obstetrics) classification for ovarian tumors. Twenty children and adolescents were classified as FIGO stage Ia; 8 children had stage Ic tumors. In 4 patients stage IIc and in 1 patient stage IIIc tumors were observed. For local tumor control all 33 patients underwent tumor resection and oophorectomy, which was complete in 28 patients. Adjuvant combination chemotherapy was used in 1 girl who presented with high mitotic pathological index features in FIGO stage Ia. In 8 other children between FIGO stage Ic and IIIc, treatment was also intensified by multidrug chemotherapy. After a follow-up period of 168 months, an event-free survival of 0.75 +/- 0.07 was observed. From our data we conclude that multidrug chemotherapy including cisplatin-based regimens may be useful to enhance treatment results of JGCT, especially in advanced FIGO stages.