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.[30,34,37,38,39]
Sertoli-Leydig cell tumors
Sertoli-Leydig cell tumors are rare in young girls but may present with virilization  or precocious puberty.[41,42] These tumors may also be associated with Peutz-Jeghers syndrome. 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.[Level of evidence: 3iiB]; [45,46][Level of evidence: 3iiiA]
Carcinoma of the Cervix and Vagina
Incidence, risk factors, and clinical presentation
Adenocarcinoma of the cervix and vagina is rare in childhood and adolescence, with fewer than 50 reported cases.[24,47] Two-thirds of the cases are related to in utero exposure to diethylstilbestrol.
The median age at presentation is 15 years, with a range of 7 months to 18 years, and with most patients presenting with vaginal bleeding. Adults with adenocarcinoma of the cervix or vagina will present with stage I or stage II disease 90% of the time. In children and adolescents, there is a high incidence of stage III and stage IV disease (24%). This difference may be explained by the practice of routine pelvic examinations in adults and the hesitancy to perform pelvic exams in children.