M. Paulussen et al., PRIMARY METASTATIC (STAGE-IV) EWING TUMOR - SURVIVAL ANALYSIS OF 171 PATIENTS FROM THE EICESS STUDIES, Annals of oncology, 9(3), 1998, pp. 275-281
Background. In the multicenter European Intergroup Cooperative Ewing's
Sarcoma Studies, localized Ewing tumors of bone were treated by combi
nation chemotherapy with surgery and/or radiotherapy. Patients with pr
imary metastases (pm-pts) were treated in high risk protocols. Patient
s and methods.. One hundred seventy-seven pm-pts were registered from
January 1990 to December 1995, 171 were evaluable for survival analyse
s. Thirty-six pm-pts received myeloablative megatherapy with stem cell
rescue following conventional treatment. Bilateral whole lung irradia
tion (WLI) was administered in 57 pm-pts with pulmonary involvement. E
vent-free survival (EFS) rates were estimated by Kaplan-Meier analysis
. Prognostic factors were identified by log-rank statistics, Cox proce
dures and logistic regression. Results. Eighty-nine deaths were record
ed by 1 February 1997, EFS four years after diagnosis for all 171 pm-p
ts was 0.27. EFS for isolated lung metastases was 0,34, for bone/bone
marrow (BM) metastases, 0,28, and for combined lung plus bone/BM metas
tases, 0.14 (P < 0.005). WLI improved outcome in case of isolated pulm
onary involvement (0.40 vs. 0.19, P < 0,05). In pm-pts with combined p
ulmonary/skeletal metastases, intensification by megatherapy and/or WL
I improved EFS from 0.00 to 0.27 (P = 0.0001). Conclusions. EFS four y
ears after diagnosis in patients with disseminated Ewing tumors is 0,2
7. Whole lung irradiation and megatherapy improve outcome in subgroups
of patients with disseminated Ewing disease.