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 ...Continue Reading
References
Apr 6, 2001·Skeletal Radiology·C S van RijswijkJ L Bloem
Jul 4, 2001·Genes, Chromosomes & Cancer·I PanagopoulosN Mandahl
Jul 10, 2002·Lancet·Brian O'SullivanBenny Zee
Aug 1, 2002·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Sheri L SpuntAlberto S Pappo
Apr 17, 2003·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Mehmet Fatih OkcuBeverly Raney
Jul 27, 2004·Cancer·Andrea FerrariPaolo G Casali
Sep 15, 2004·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Louis GuillouJean-Michel Coindre
Sep 24, 2004·Pediatric Blood & Cancer·Arnold C PaulinoB-Chen Wen
May 10, 2005·Expert Review of Anticancer Therapy·Andrea Ferrari, Michela Casanova
Aug 2, 2008·The Journal of Bone and Joint Surgery. British Volume·F ChotelR J Grimer
Dec 18, 2008·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Robert J CanterSamuel Singer
May 20, 2009·Cancer·Emanuela PalmeriniSefano Ferrari
Jun 11, 2009·Cancer·Iyad SultanAndrea Ferrari
Jul 14, 2009·European Journal of Nuclear Medicine and Molecular Imaging·Mathieu CharestRobert E Turcotte
Oct 27, 2009·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Stefan SleijferJean-Yves Blay
Jun 29, 2010·Nature Medicine·Frédéric ChibonAlain Aurias
Jul 1, 2010·Pediatric Blood & Cancer·Cristina MeazzaAndrea Ferrari
Apr 16, 2011·Pediatric Blood & Cancer·Daniel OrbachMike C Stevens
May 10, 2011·International Journal of Radiation Oncology, Biology, Physics·Kristy B SmithRobert B Marcus
Jul 28, 2011·Pediatric Blood & Cancer·Andrea FerrariSheri L Spunt
Feb 11, 2012·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Andrea FerrariGianni Bisogno
Jul 28, 2012·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Andrea FerrariGianni Bisogno
Jan 16, 2013·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Pauline LagardeFrédéric Chibon
Feb 12, 2014·European Journal of Cancer : Official Journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR)·Jean-Yves BlaySant P Chawla
Mar 19, 2014·Journal of Pediatric Hematology/oncology·Anna KerouantonDaniel Orbach
Mar 26, 2014·Pediatric Blood & Cancer·Frederick SooleDaniel Orbach
Apr 26, 2014·Journal of Cancer Research and Therapeutics·Yetisyigit TarkanGumus Mahmut
Apr 29, 2014·Medical Oncology·Sameer YaserAbdelatif Al-Mousa
May 21, 2014·The International Journal of Biochemistry & Cell Biology·Joanna PrzybylMaria Debiec-Rychter
Jul 30, 2014·Annals of Oncology : Official Journal of the European Society for Medical Oncology·C ChakibaF Chibon
Dec 2, 2014·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·S Papillard-MaréchalD Orbach
Dec 10, 2014·Annals of Oncology : Official Journal of the European Society for Medical Oncology·A FerrariD Orbach