Juvenile granulosa cell tumors
The most common histologic subtype in girls younger than 18 years is juvenile granulosa cell tumors (median age, 7.6 years; range, birth to 17.5 years).[33,34] Juvenile granulosa cell tumors represent about 5% of ovarian tumors in children and adolescents and are distinct from the granulosa cell tumors seen in adults.[31,35,36,37]
Most patients with juvenile granulosa cell tumors present with precocious puberty. Other presenting symptoms include abdominal pain, abdominal mass, and ascites. Juvenile granulosa cell tumors has been reported in children with Ollier disease and Maffucci syndrome.
As many as 90% of children with juvenile granulosa cell tumors will have low-stage disease (International Federation of Gynecology and Obstetrics [FIGO] stage I) and are usually curable with unilateral salpingo-oophorectomy alone. Patients with advanced disease (FIGO stage II–IV) and those with high mitotic activity tumors have a poorer prognosis.
Use of a cisplatin-based chemotherapy regimen has been reported in both the adjuvant and recurrent disease settings with some success.[33,37,40,41,42]
Sertoli-Leydig cell tumors
Sertoli-Leydig cell tumors are rare in young girls but may present with virilization  or precocious puberty.[44,45] These tumors may also be associated with Peutz-Jeghers syndrome. A Chinese group reported on 40 women with FIGO stage I or IC Sertoli-Leydig cell tumors of the ovary, with an average age of 28 years.[Level of evidence: 3iiA] Of 34 patients with intermediate or poor differentiation, 23 received postoperative chemotherapy (most regimens included cisplatin); none recurred. Of the 11 patients who did not receive postoperative chemotherapy, two recurred; both were salvaged with chemotherapy. In contrast to juvenile granulosa cell tumors, a recent study suggested that Sertoli-Leydig tumors with abdominal spillage (FIGO stage IC) should be treated with cisplatin-based chemotherapy.
Small cell carcinoma of the ovary
Small cell carcinomas of the ovary are exceedingly rare and aggressive tumors and may be associated with hypercalcemia. Successful treatment with aggressive therapy has been reported in a few cases.[48,49][Level of evidence: 3iiB]; [50,51][Level of evidence: 3iiiA]