[Specificities of sex-cord XXXX in @entity1 and adolescents].

@entity1 @entity5 @entity4 @entity900 @entity247 @entity11597 @entity2588 @entity23718
UNASSIGNED: @entity11597 ( @entity11597 ) are rare @entity5 accounting for less than 5% of @entity5 in @entity1 and adolescents. They differ from those diagnosed in adults by their presentation, histology, evolution and treatment modalities. @entity11597 occur mostly in @entity1 less than 6 months. @entity4 is often the only symptom, but signs of hormonal secretion with @entity247 may be present. Juvenile granulosa @entity11597 is the main histologic subtype. Sertoli SCTs are much less frequent while Leydig @entity5 occurred in older @entity1 and adolescents. Prognosis is excellent after inguinal orchiectomy. Testis sparing surgery could be performed but indications and modalities have to be strongly defined. Ovarian @entity11597 are diagnosed in older @entity1 and adolescents and present with abdominal symptoms and/or signs of hormonal secretion: estrogenic manifestations (isosexual pseudoprecocity, menometrorrhagia) or @entity23718 (hirsutism, @entity2588 ). Main histologic subtype is juvenile granulosa (rarely Sertoli-Leydig). If oophorectomy (or salpingo-oophorectomy) may be curative for localized disease, adjuvant @entity900 -containing chemotherapy is mandatory in case of @entity5 rupture or peritoneal dissemination to prevent recurrences. Because of the rarity of these @entity5 , concerted multidisciplinary cares are required to best adapt therapeutic strategy before any surgical intervention.
