title: Primary Ewing Family of Tumors of the Jaw Has a Better Prognosis Compared to Tumors of Extragnathic Sites.
abstract: OBJECTIVE
  Primary Ewing sarcoma of the jaw is rare. The aim of this study was to describe new cases of primary Ewing sarcoma of the jaw and investigate reported prognostic factors of Ewing sarcoma in this series and treatment outcome.
  
  
  METHODS
  Six patients with primary Ewing sarcoma of the jaw were treated at the Memorial Sloan Kettering Cancer Center (MSKCC) from 1992 through 2013. Clinical data, pathology reports, treatment prescribed, treatment regimens, outcome, and follow-up information were reviewed.
  
  
  RESULTS
  Five of 6 patients were female and 5 cases were in the mandible. No patient presented with metastatic disease at diagnosis. All cases were positive for CD99, and 3 patients with genetic confirmation were positive for EWS-FLI1 fusion or EWSR1 gene rearrangement. All patients received induction multiagent chemotherapy and surgical resection and 2 patients received adjuvant radiotherapy. Total (grade IV) or nearly total (grade III) tumor necrosis in 3 of 5 patients (60%) assessed for histologic response to chemotherapy indicated intense sensitivity. All patients were alive and free of disease, with no history of local recurrence, at a median follow-up period of 6.5 years.
  
  
  CONCLUSIONS
  Patients with primary Ewing sarcoma of the jaw have a good prognosis and metastasis is an uncommon occurrence at initial presentation.
authors.affiliation: Dental Oncology Research Fellow, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Adepitan A
authors.initials: AA
authors.lastname: Owosho
authors.affiliation: Former Research Fellow, Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY; Clinical Assistant Professor, School of Dentistry, University of Michigan, Ann Arbor, MI.
authors.forename: Eugene
authors.initials: E
authors.lastname: Ko
authors.affiliation: Research Study Assistant, Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Haley I
authors.initials: HI
authors.lastname: Rosenberg
authors.affiliation: Assistant Attending, Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: SaeHee K
authors.initials: SK
authors.lastname: Yom
authors.affiliation: Attending, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.affiliation: Chief, Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Huryn
authors.affiliation: Attending, Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY. Electronic address: estiloc@mskcc.org.
authors.forename: Cherry L
authors.initials: CL
authors.lastname: Estilo
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007573:Jaw Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult