title: [Synovial sarcoma in children and adolescents].
abstract: Synovial sarcoma (SS) is a rare high-grade malignant mesenchymal tumor affecting children, adolescents, and young adults. Cytogenetically, more than 90% of SS is characterized by the t(X;18)(p11.2;q11.2), translocation resulting in two chimeric fusion genes SYT-SSX1 and SYT-SSX2, confirming histological diagnosis. Pediatric SS arises most often in soft tissues of the extremities (66% of cases), and is a localized tumor without spreading to regional lymph nodes (96% of cases) nor to metastatic sites (94% of cases). Although clinical and radiologic presentation, histologic analysis and tumor biology appear similar in pediatric and adolescent SS, outcome seems better in children than in adolescents, respectively 84% vs 60% of 5years overall survival (OS). If complete resection is the gold standard in SS, other therapeutic modalities differ between pediatric and adult populations, considering SS as an intermediate chemosensitive tumor more frequently by pediatric oncologists. Prognostic factors evaluation (tumor size, site of primary and IRS group) is necessary to establish optimal treatment strategies, with multimodal therapeutic approach in children and adolescents. Thus, recent results about the European prospective EpSSG NRSTS 05 study for children and adolescent patients with localized SS showed a 5years OS >90%. Moreover, recent somatic genetic data about SS open the debate on an appropriate strategy based and stratified on tumor genomic. Multinational prospective pediatric, adolescent and young adult study is necessary to improve optimal and appropriate approach in this rare tumor.
authors.affiliation: CHU de Nancy, hôpital mère-enfant, service d'oncologie pédiatrique, 54000 Nancy, France.
authors.forename: Ludovic
authors.initials: L
authors.lastname: Mansuy
authors.affiliation: Institut de cancérologie de Lorraine, département de radiothérapie, 54500 Vandœuvre-lès-Nancy, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Bernier
authors.affiliation: Centre Léon-Bérard, département d'anatomopathologie, 69673 Lyon, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Ranchère-Vince
authors.affiliation: CHU de Nancy, hôpital mère-enfant, département de radiologie, 54000 Nancy, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Mainard
authors.affiliation: Institut Curie, département de pédiatrie, adolescents et jeunes adultes, 75005 Paris, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.affiliation: CHU de Nantes, hôpital mère-enfant, service d'oncologie pédiatrique, 7, quai Moncousu, 44093 Nantes, France. Electronic address: nadege.corradini@chu-nantes.fr.
authors.forename: Nadège
authors.initials: N
authors.lastname: Corradini
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015321:Gene Rearrangement; D006801:Humans; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011379:Prognosis; D035583:Rare Diseases / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy; D013584:Sarcoma, Synovial / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000628:therapy; D047368:Tumor Burden
chemical_list: D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein
keywords: Adolescents; Children; Enfants; Facteurs pronostiques; Prognostic factors; SYT-SSX; Sarcoma; Sarcomes; Synovial sarcomas; Synovialosarcomes