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

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Most patients with juvenile granulosa cell tumors present with precocious puberty.[35] 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.[36]

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 [40] or precocious puberty.[41,42] These tumors may also be associated with Peutz-Jeghers syndrome.[43] 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.[39]

Small cell carcinoma of the ovary

Small cell carcinomas of the ovary are exceedingly rare and aggressive tumors and may be associated with hypercalcemia.[44] Successful treatment with aggressive therapy has been reported in a few cases.[44][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.

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