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    Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Genital / Urinary Tumors

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    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.[38] 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.[39]

    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 [43] or precocious puberty.[44,45] These tumors may also be associated with Peutz-Jeghers syndrome.[46] 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.[47][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.[42]

    Small cell carcinoma of the ovary

    Small cell carcinomas of the ovary are exceedingly rare and aggressive tumors and may be associated with hypercalcemia.[48] Successful treatment with aggressive therapy has been reported in a few cases.[48,49][Level of evidence: 3iiB]; [50,51][Level of evidence: 3iiiA]

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