title: Ewing sarcoma/primitive neuroectodermal tumor of the chest wall: impact of initial versus delayed resection on tumor margins, survival, and use of radiation therapy.
abstract: OBJECTIVE
  To establish outcome and optimal timing of local control for patients with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) of the chest wall.
  
  
  METHODS
  Patients < or =30 years of age with ES/PNET of the chest wall were entered in 2 consecutive protocols. Therapy included multiagent chemotherapy; local control was achieved by resection, radiotherapy, or both. We compared completeness of resection and disease-free survival in patients undergoing initial surgical resection versus those treated with neoadjuvant chemotherapy followed by resection, radiotherapy, or both. Patients with a positive surgical margin received radiotherapy.
  
  
  RESULTS
  Ninety-eight (11.3%) of 869 patients had primary tumors of the chest wall. Median follow-up was 3.47 years and 5-year event-free survival was 56% for the chest wall lesions. Ten of 20 (50%) initial resections resulted in negative margins compared with 41 of 53 (77%) negative margins with delayed resections after chemotherapy (P = 0.043). Event-free survival did not differ by timing of surgery (P = 0.69) or type of local control (P = 0.17). Initial chemotherapy decreased the percentage of patients needing radiation therapy. Seventeen of 24 patients (70.8%) with initial surgery received radiotherapy compared with 34 of 71 patients (47.9%) who started with chemotherapy (P = 0.061). If a delayed operation was performed, excluding those patients who received only radiotherapy for local control, only 25 of 62 patients needed radiotherapy (40.3%; P = 0.016).
  
  
  CONCLUSIONS
  The likelihood of complete tumor resection with a negative microscopic margin and consequent avoidance of external beam radiation and its potential complications is increased with neoadjuvant chemotherapy and delayed resection of chest wall ES/PNET.
authors.affiliation: Department of Surgery, Children's Hospital Boston, MA 02115, USA. Robert.Shamberge@TCH.Harvard.edu
authors.forename: Robert C
authors.initials: RC
authors.lastname: Shamberger
authors.forename: Michael P
authors.initials: MP
authors.lastname: LaQuaglia
authors.forename: Mark C
authors.initials: MC
authors.lastname: Gebhardt
authors.forename: James R
authors.initials: JR
authors.lastname: Neff
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Tarbell
authors.forename: Karen C
authors.initials: KC
authors.lastname: Marcus
authors.forename: Scott L
authors.initials: SL
authors.lastname: Sailer
authors.forename: Richard B
authors.initials: RB
authors.lastname: Womer
authors.forename: James S
authors.initials: JS
authors.lastname: Miser
authors.forename: Paul S
authors.initials: PS
authors.lastname: Dickman
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Perlman
authors.forename: Meenakshi
authors.initials: M
authors.lastname: Devidas
authors.forename: Stephen B
authors.initials: SB
authors.lastname: Linda
authors.forename: Mark D
authors.initials: MD
authors.lastname: Krailo
authors.forename: Holcombe E
authors.initials: HE
authors.lastname: Grier
authors.forename: Linda
authors.initials: L
authors.lastname: Granowetter
mesh_terms: D000328:Adult; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D006801:Humans; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery*; D016032:Randomized Controlled Trials as Topic; D012272:Ribs; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery*; D013997:Time Factors