title: Management and Outcome of Ewing Sarcoma of the Head and Neck.
abstract: BACKGROUND
  Ewing sarcoma (EWS) of the head and neck is rare. Multimodal treatment consists of chemotherapy and local treatment; however, local treatment for EWS of the head and neck is challenging. The first objective was to describe local treatment administered to the patients with localized EWS of the head and neck according to the EURO-E.W.I.N.G.99-trial, and to assess the impact on survival. The second objective was to systematically review the scientific literature available for this topic.
  
  
  METHODS
  Fifty-one patients were included. Local control consisted of surgery and/or radiotherapy (RT). Event-free survival (EFS) and overall survival (OS) were determined. Outcome was analyzed by comparing local treatment approaches. A Medline search was performed for EWS of the head and neck.
  
  
  RESULTS
  Eighty-six percent of patients had localized disease. Most common primary sites included the skull (45%), maxilla (14%), and mandible (12%). Three-year EFS was 74% and 3-year OS was 87% for patients with localized disease. EFS was 40% for patients >15 years compared to 81% for patients <15 years. Local control consisted of surgery (S; 33%), RT (18%), or S + RT (45%). Related 3-year EFS was 81% (S), 80% (RT), and 72% (S + RT); 3-year OS was 80%, 76%, and 81%, respectively.
  
  
  CONCLUSIONS
  In patients with EWS of the head and neck, age, and stage are important prognostic factors. Although not statistically significant, large tumor volume seems to be a negative prognostic factor. No difference in EFS and OS could be found when comparing patients treated with surgery, RT, or combined surgery and RT.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany.
authors.forename: Knut
authors.initials: K
authors.lastname: Grevener
authors.affiliation: Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Lianne M
authors.initials: LM
authors.lastname: Haveman
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Ranft
authors.affiliation: Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Henk
authors.initials: H
authors.lastname: van den Berg
authors.affiliation: Department of Maxillofacial Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Jung
authors.affiliation: St Anna Children's Hospital, Vienna, Austria.|Children's Cancer Research Institute, Vienna, Austria.
authors.forename: Ruth
authors.initials: R
authors.lastname: Ladenstein
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Klco-Brosius
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany.
authors.forename: Heribert
authors.initials: H
authors.lastname: Juergens
authors.affiliation: Department of Maxillofacial Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: J Hans M
authors.initials: JH
authors.lastname: Merks
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital Muenster, Muenster, Germany.
authors.forename: Uta
authors.initials: U
authors.lastname: Dirksen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols; D001859:Bone Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods*; D018572:Disease-Free Survival; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D019637:Orthopedic Procedures; D016016:Proportional Hazards Models; D011878:Radiotherapy; D018714:Radiotherapy, Adjuvant / Q000379:methods; D012307:Risk Factors; D012512:Sarcoma, Ewing / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
keywords: Ewing sarcoma; head & neck; outcome; treatment