Ewing sarcoma/primitive neuroectodermal tumor of the chest wall: impact of initial versus delayed resection on tumor margins, survival, and use of radiation therapy.
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.
Shamberger
Robert C
RC
Department of Surgery, Children's Hospital Boston, MA 02115, USA. Robert.Shamberge@TCH.Harvard.edu
LaQuaglia
Michael P
MP
Gebhardt
Mark C
MC
Neff
James R
JR
Tarbell
Nancy J
NJ
Marcus
Karen C
KC
Sailer
Scott L
SL
Womer
Richard B
RB
Miser
James S
JS
Dickman
Paul S
PS
Perlman
Elizabeth J
EJ
Devidas
Meenakshi
M
Linda
Stephen B
SB
Krailo
Mark D
MD
Grier
Holcombe E
HE
Granowetter
Linda
L
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