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Synovial Sarcoma in Children and Adolescents

Kerouanton, Anna MD; Jimenez, Irene MD; Cellier, Cecile MD; Laurence, Valerie MD; Helfre, Sylvie MD; Pannier, Stephanie MD; Mary, Pierre MD; Freneaux, Paul MD; Orbach, Daniel MD

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0000000000000154
Invited Review Article
Abstract

Synovial sarcoma (SS) is a high-grade soft tissue sarcoma characterized by local invasiveness and a propensity to metastasize, affecting pediatric, adolescent, and adult populations. The peak incidence is observed in the third decade of life and SS is the most common nonrhabdomyosarcoma soft tissue sarcoma in childhood and adolescence. Although pediatric and adult SS appear clinically and radiologically identical, treatment modalities may differ according to the patient’s age. For many years, pediatric oncologists have treated SS as a chemosensitive tumor according to the “rhabdomyosarcoma philosophy.” In contrast, adult oncologists generally treat this tumor as a poorly chemosensitive tumor and focus on local control. The authors propose an update of SS in the pediatric population and analyze their results to those obtained in adults.

Author Information

*Pediatric, Adolescent and Young Adult Department

Departments of Radiology

Medical Oncology

#Pathology

§Radiotherapy Department, Institut Curie

Pediatric Orthopedic Department, Necker Hospital—Assistance Publique, Université Paris Descartes—Sorbonne Paris

Pediatric Orthopedic Department, Armand Trousseau Hospital—Assistance Publique, Paris, France

The authors declare no conflict of interest.

Reprints: Daniel Orbach, MD, Pediatric Adolescent and Young Adult Department, Institut Curie, 26 rue d’Ulm, Paris 75005, France (e-mail: daniel.orbach@curie.fr).

Received January 22, 2014

Accepted February 19, 2014

Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.