10040 Background: Osteosarcoma is the most frequent bone tumor in children and adolescents. The aim of this study is to evaluate the demographic characteristics, therapy, and long term outcome of children with osteosarcoma in a single institution. Methods: Between January 1990 and December 2006, 94 children (53 male, 41 female) with a median age of 13 (5–16) years and a histopathologic diagnosis of osteosarcoma were treated with an institutional chemotherapy regimen comprising of 6 courses (3 pre-, 3 postoperatively) of epirubicin (90 mg/m2), cisplatin(100 mg/m2), and ifosfamide(2 g/m2 × 3 days) every 3 weeks. Results: The median follow-up time was 36 (2–219) months. 68 patients were alive at the time of analysis. 26 patients died; 20 of disease, 5 of toxicity, and 1 of second malignancy (acute myeloid leukemia). 90% of the patients had a limb salvage. 5 and 10 year OS for all patients were 64.7 % ( [95 % CI] 74.8–52.94 %) and 62.2 % (95 % CI 74.6–49.9 %), respectively. 5 and 10 year EFS for all patients was 51.8 % (95% CI 40.2–63.4 %). 5 and 10 year OS for nonmetastatic patients were 78.3 % (95% CI 66.9–89.7 %) and 75.1 %(95% CI 62.6–87.6), vs. 13.5 % 5 year OS for metastatic patients(95% CI 0–30.8 %) (p< 0.001). 5 and 10 year EFS for nonmetastatic patients was superior to those with metastatic disease [62.4 % (95% CI 49.9–79.9 %) vs. 6.9 % (95% CI 0–19.9 %)) (p<0.001). A total of 33 patients experienced relapse and/or progression at a median of 9 months (range 0–40 months). 10 year OS for 18 patients (11 metastatic at diagnosis) who progressed during preoperative chemotherapy was 13 % vs. 75 % for those who didnot have progressive disease (p< 0.001). The rate of histologically good response to preoperative chemotherapy was 64.5 %. Histologic response (<90 percent necrosis vs ≥90 percent) significantly correlated with 5 year EFS (31 % vs 67.6 %, p=0.023) but not with OS (57.7 % vs 76.5 %, p=0.13). Conclusions: The presence of metastases at diagnosis was the most significant characteristic influencing outcome. Our results demonstrate that the combination of epirubicin, cisplatin, and ifosfamide is an active and reasonably well-tolerated regimen for childhood osteosarcoma. No significant financial relationships to disclose.