title: [Specificities of sex-cord stromal tumors in children and adolescents].
abstract: Sex-cord stromal tumors (SCT) are rare pediatric tumors accounting for less than 5% of gonadal tumors in children and adolescents. They differ from those diagnosed in adults by their presentation, histology, evolution and treatment modalities. Testicular SCT occur mostly in infants less than 6 months. Testicular swelling is often the only symptom, but signs of hormonal secretion with gynecomastia may be present. Juvenile granulosa SCT is the main histologic subtype. Sertoli SCTs are much less frequent while Leydig tumors occurred in older children and adolescents. Prognosis is excellent after inguinal orchiectomy. Testis sparing surgery could be performed but indications and modalities have to be strongly defined. Ovarian SCT are diagnosed in older children and adolescents and present with abdominal symptoms and/or signs of hormonal secretion: estrogenic manifestations (isosexual pseudoprecocity, menometrorrhagia) or virilization (hirsutism, amenorrhea). Main histologic subtype is juvenile granulosa (rarely Sertoli-Leydig). If oophorectomy (or salpingo-oophorectomy) may be curative for localized disease, adjuvant cisplatin-containing chemotherapy is mandatory in case of tumor rupture or peritoneal dissemination to prevent recurrences. Because of the rarity of these pediatric tumors, concerted multidisciplinary cares are required to best adapt therapeutic strategy before any surgical intervention.
authors.affiliation: CHU, service d'hématologie et oncologie pédiatrique, 44000 Nantes, France.
authors.forename: Estelle
authors.initials: E
authors.lastname: Thebaud
authors.affiliation: Institut Curie, département de pédiatrie-adolescents-jeunes adultes, 75005 Paris, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.affiliation: Groupement hospitalier Est du CHU de Lyon, hôpital Femme Mère-Enfant, service de chirurgie pédiatrique, 69500 Bron, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Faure-Conter
authors.affiliation: Gustave-Roussy, département de cancérologie de l'enfant et de l'adolescent, 94805 Villejuif, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Patte
authors.affiliation: Institut d'hémato-oncologie pediatrique, service de chirurgie pédiatrique, 69008 Lyon, France.
authors.forename: Frederic
authors.initials: F
authors.lastname: Hameury
authors.affiliation: CHU, hôpital Lapeyronie, service de chirurgie viscérale et urologique pédiatrique, 34295 Montpellier, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Kalfa
authors.affiliation: Groupement hospitalier Est du CHU de Lyon, hôpital Femme Mère-Enfant, centre de pathologie Est, 69500 Bron, France.
authors.forename: Frédérique
authors.initials: F
authors.lastname: Dijoud
authors.affiliation: CHU de Bicêtre, université Paris XI, service de chirurgie pédiatrique, 94270 Le Kremlin-Bicêtre, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Martelli
authors.affiliation: Gustave-Roussy, département de cancérologie de l'enfant et de l'adolescent, 94805 Villejuif, France. Electronic address: brice.fresneau@gustaveroussy.fr.
authors.forename: Brice
authors.initials: B
authors.lastname: Fresneau
mesh_terms: D000293:Adolescent; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D020022:Genetic Predisposition to Disease; D006106:Granulosa Cell Tumor / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D009386:Neoplastic Syndromes, Hereditary; D010051:Ovarian Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D024162:Second-Look Surgery; D018312:Sex Cord-Gonadal Stromal Tumors / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy; D013736:Testicular Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery; D014463:Ultrasonography
keywords: Adolescent; Children; Enfant; Ovarian tumor; Sex-cordially; Stromal tumors; Testicular tumor; Tumeur ovarienne; Tumeur testiculaire; Tumeurs des cordons sexuels