Ewing's sarcoma and primitive neuroectodermal tumor of hand and forearm. Experience of the Cooperative Ewing's Sarcoma Study Group.
OBJECTIVE
Because Ewing's sarcoma (EWS) is extremely rare in the hand and forearm, only limited data are available for planning treatment or predicting prognosis.


METHODS
Data of 33 patients with EWS of the forearm or hand who were enrolled in studies of the German Association for Paediatric Oncology/Haematology and the European Intergroup Cooperative Ewing's Sarcoma Study Group were analyzed. Patients received neoadjuvant multi-agent chemotherapy according to the valid protocol. Local treatment consisted of surgery (n=7), radiotherapy (n=7), or a combination of both (n=19).


RESULTS
The 5/10-year overall survival rate was 84.1% (95% CI: 71.2-96.9)/74.1% (95% CI: 56.8-91.5), and both 5/10-year event-free survival rate were 71.3% (95% CI: 55.4-87.1). Only one of seven patients with secondary metastases was in remission at the time of analysis. One patient with local recurrence and another with primary metastases died. Altogether, eight of 33 patients died of their disease. The event-free survival rate was 80.6% in patients with good response to chemotherapy and 33.3% in patients with poor response. Surgery in combination with radiotherapy achieved a higher survival rate compared with radiotherapy or surgery alone. One out of two patients with non-wide margins of resection died of disease.


CONCLUSIONS
The results demonstrate a remarkably high survival rate for patients with EWS of the hand and forearm.
Daecke
Wolfgang
W
Orthopädische Universitätsklinik Heidelberg, Schlierbacher Landstrasse 200, 69118 Heidelberg, Germany. wolfgang.daecke@ok.uni-heidelberg.de
Ahrens
Susanne
S
Juergens
Herbert
H
Martini
Abdul-Kader
AK
Ewerbeck
Volker
V
Kotz
Rainer
R
Winkelmann
Winfried
W
Bernd
Ludger
L
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000473:pathology / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002986:Clinical Trials as Topic; D005260:Female; D005858:Germany; D006225:Hand; D006801:Humans; D008297:Male; D015337:Multicenter Studies as Topic; D020360:Neoadjuvant Therapy / Q000379:methods; D018242:Neuroectodermal Tumors, Primitive / Q000556:secondary / Q000628:therapy*; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D011884:Radius; D012189:Retrospective Studies; D012307:Risk Factors; D012512:Sarcoma, Ewing / Q000556:secondary / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome; D014457:Ulna