Objective: To test whether patients with Askin tumor treated with aggr
essive neoadjuvant chemotherapy have a better clinical outcome. Design
: Retrospective case series. Setting: Pediatric referral center. Patie
nts: All children diagnosed with malignant small-cell tumors of the ch
est wall (Askin tumor) and treated from 1975 to September 1987 (phase
1, n = 6) and from September 1987 to the present (phase 2, n = 9). Mai
n Outcome Measures: Survival as a function of extent of disease and re
sponse to therapy as measured by tumor volume, survival, and recurrenc
e. Results: All phase 2 patients had significant reduction of tumor vo
lume and improved survival by Kaplan-Meier estimates compared with pha
se 1 patients. No phase 1 patients are still alive. Conclusion: Patien
ts with Askin tumor treated with aggressive preresection chemotherapy
have smaller tumors to resect and improved survival.