title: Nonparameningeal head and neck rhabdomyosarcoma in children and adolescents: Lessons from the consecutive International Society of Pediatric Oncology Malignant Mesenchymal Tumor studies.
abstract: This article reports risk factors and long-term outcome in localized nonparameningeal head and neck rhabdomyosarcomas in children and adolescents from a combined dataset from 3 consecutive international trials.
  
  
  
  Data from 140 children (9.3% of total) prospectively enrolled in the International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT)-84/89/95 studies were analyzed.
  
  
  
  Primary site was: superficial face in 46%; oral cavity (21%); neck (19%); and salivary glands (14%). Local control was achieved in 96%, but 49% relapsed (locoregionally 91%). At median follow-up of 10 years, 5-year overall survival (OS) was 74.7% (67.4% to 81.9%) and event-free survival 48.9% (40.6% to 57.2%), although this improved with successive studies. Radiotherapy (RT) as first-line treatment was independently prognostic for event-free survival (relative risk [RR] = 0.4 [range, 0.2-0.7]; p < .01) even if it did not impact OS (RR = 1 [range, 0.5-2]).
  
  
  
  High rates of locoregional relapse were seen in head and neck rhabdomyosarcoma that should be prevented by more frequent use of RT in this primary. © 2016 Wiley Periodicals, Inc. Head Neck 39: 24-31, 2017.
authors.affiliation: Adolescent and Pediatric Oncology Department, Institut Curie, Paris, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.affiliation: Department of Biostatistics, Institut Curie, Paris, France.
authors.forename: Veronique
authors.initials: V
authors.lastname: Mosseri
authors.affiliation: Paediatric Oncology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
authors.forename: Soledad
authors.initials: S
authors.lastname: Gallego
authors.affiliation: Pathology Department, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors.forename: Anna
authors.initials: A
authors.lastname: Kelsey
authors.affiliation: Pediatric Haematology Oncology Department, Hopital Universitaire des enfants, Brussels, Belgium.
authors.forename: Christine
authors.initials: C
authors.lastname: Devalck
authors.affiliation: Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors.forename: Bernadette
authors.initials: B
authors.lastname: Brenann
authors.affiliation: Center of Pediatric Oncology, Princess Máxima, Utrecht, The Netherlands.
authors.forename: Max M
authors.initials: MM
authors.lastname: van Noesel
authors.affiliation: Department of Pediatric Oncology, Institut d'Hematologie et d'Oncologie Pédiatrique, Lyon, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.affiliation: Department of Pediatric Oncology, Emma Children's Hospital - Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Johannes H M
authors.initials: JH
authors.lastname: Merks
authors.affiliation: Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark.
authors.forename: Catherine
authors.initials: C
authors.lastname: Rechnitzer
authors.affiliation: Department of Pediatric Oncology, Children's Hospital for Wales, Heath Park, Cardiff, United Kingdom.
authors.forename: Meriel
authors.initials: M
authors.lastname: Jenney
authors.affiliation: Pediatric Oncology Unit, Gustave-Roussy, Villejuif, France.
authors.forename: Veronique
authors.initials: V
authors.lastname: Minard-Colin
authors.affiliation: School of Clinical Sciences, University of Bristol, Bristol, United Kingdom.
authors.forename: Michael
authors.initials: M
authors.lastname: Stevens
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008637:Mesenchymoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome
keywords: International Society of Pediatric Oncology Malignant Mesenchymal Tumor (SIOP-MMT) protocols; children; head and neck rhabdomyosarcoma; radiotherapy; surgery