title: [Alveolar soft part sarcoma in pediatric patients].
abstract: Alveolar soft part sarcoma, ASPS, is a rare malignant tumor, with preferential primary localization in limbs, usually occurring in adolescents and young adults. This sarcoma, well defined histologically and at molecular level, has an indolent course, but a high potential metastatic pulmonary and cerebral evolution, sometimes late. ASPS is characterized by an almost specific translocation t(X, 17)(p11;25) which creates a fusion protein, APSL-TFE3, acting as an aberrant transcription factor. An in-bloc resection of the primary tumor is the treatment of choice in cases of localized disease. Conventional chemotherapy is generally ineffective. The role of radiotherapy is discussed in case of micro- or macroscopical incomplete residue. It seems to reduce local recurrence, but did not influence overall survival. The 5 years survival rate in children, adolescents and young adults is close to 80% in case of localized disease but poorer in presence of metastases. Recently, systemic anti-tumoral treatments have been focused on the use of targeted therapies. Anti-angiogenic drugs and tyrosine kinase inhibitors are the most promising approaches, but require further study. Prognostic risk factors in the literature are age (>10Y), tumor size (>5cm) and presence of metastases. This article reviews the clinical manifestations, diagnosis modalities, radiographic characteristics and therapeutic strategy of this disease in the pediatric population.
authors.affiliation: Hôpital Hautepierre, service d'hématologie et d'oncologie pédiatrique, avenue Molière, 67098 Strasbourg, France; Inserm UMR-S1109, université de Strasbourg, laboratoire d'immuno-rhumatologie moléculaire, 67098 Strasbourg, France. Electronic address: catherine.paillard@chru-strasbourg.fr.
authors.forename: Catherine
authors.initials: C
authors.lastname: Paillard
authors.affiliation: Hôpital Armand-Trousseau-Assistance publique, service d'anatomie pathologique, 26, avenue du Dr-Arnold-Netter, 75012 Paris, France.
authors.forename: Aurore
authors.initials: A
authors.lastname: Coulomb
authors.affiliation: Institut Curie, service de radiothérapie, 26, rue d'Ulm, 75005 Paris, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Helfre
authors.affiliation: Institut Curie, département d'oncologie pédiatrique adolescent jeune adulte, 26, rue d'Ulm, 75005 Paris, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D011379:Prognosis; D035583:Rare Diseases / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012307:Risk Factors; D018234:Sarcoma, Alveolar Soft Part / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy; D047368:Tumor Burden; D055815:Young Adult
keywords: Adolescent; Alveolar soft part sarcoma; Children; Enfant; Sarcome alvéolaire des parties molles; Target therapy; Thérapeutique ciblée