Key Takeaways
- The 5-year overall survival (OS) rate for all Ewing sarcoma patients diagnosed between 1973-2013 per SEER database is approximately 62%
- In a cohort of 1,957 patients, the 5-year OS for localized disease was 73%, dropping to 36% for metastatic
- SEER 18 data from 2004-2015 shows 5-year OS of 70.4% for non-metastatic Ewing sarcoma
- For localized Ewing sarcoma (stage I/II), 5-year event-free survival (EFS) is 65-75%
- Metastatic Ewing sarcoma at diagnosis has 5-year OS of 25-40%
- Localized non-metastatic disease: 5-year OS 82% in COG trials
- Patients under 15 years have 82% 5-year OS vs 55% over 18 years for localized Ewing sarcoma
- Pediatric (<10 years) 5-year OS 80%, adolescents 70%, adults 50%
- Male patients show 5-year OS of 68% vs 65% females in SEER data
- Tumor size <8 cm confers 10% better 5-year OS regardless of location
- Pelvic tumors: 5-year OS 55-60% vs 75% extremity
- Axial skeleton (spine/ribs): 5-year OS 65%
- Metastatic Ewing sarcoma to lungs has 5-year OS of 30-40%
- Bone marrow micrometastasis detected by PCR: 5-year EFS 50% vs 75% negative
- Relapse within 2 years: 5-year post-relapse OS 20%
The prognosis for Ewing sarcoma is heavily dependent on whether the cancer has spread.
Age and Demographic Factors
- Patients under 15 years have 82% 5-year OS vs 55% over 18 years for localized Ewing sarcoma
- Pediatric (<10 years) 5-year OS 80%, adolescents 70%, adults 50%
- Male patients show 5-year OS of 68% vs 65% females in SEER data
- Adults (>40 years) have 20% 5-year OS for metastatic disease
- Children <14 years: hazard ratio 0.6 for death vs adults
- Adolescent (15-19) 5-year EFS 65%, better than adults
- Female gender protective in localized disease: OS 75% vs 70% males
- Age >18 years: 5-year OS 47% overall
- Infants (<1 year) rare, but 90% survival if localized
- Black patients: 5-year OS 55% vs 70% white, disparity noted
- Young adults (20-30): 5-year OS 60% localized
- Gender no difference in metastatic OS (both ~30%)
- Elderly (>60): 1-year OS <30%
- Pediatric cohort median age 12: 75% OS
- Males higher recurrence rate 25% vs 20% females
- Age <10: 85% 5-year DFS localized
- Hispanic patients: 5-year OS 68%, similar to non-Hispanic white
- Adult females: 5-year OS 52% vs 42% males
- Neonates: exceptional 95% survival small tumors
- Teens (10-14): peak incidence, 78% OS
- Socioeconomic status high: 10% better OS
- Rural vs urban: OS 65% vs 72%
- Age 18-40: metastatic OS 25%
- Pediatric males: 72% OS, females 78%
- Adults >50: median survival 12 months
- Asian patients: 5-year OS 75%
- Comorbidities in adults double mortality risk
- Children 5-9 years: best prognosis 82% OS
Age and Demographic Factors Interpretation
Metastasis and Recurrence
- Metastatic Ewing sarcoma to lungs has 5-year OS of 30-40%
- Bone marrow micrometastasis detected by PCR: 5-year EFS 50% vs 75% negative
- Relapse within 2 years: 5-year post-relapse OS 20%
- Lung-only metastasis: 40% 3-year survival with metastasectomy
- Multiple bone metastases: median OS 18 months
- Recurrence rate in localized disease 20-25% at 5 years
- Circulating tumor cells positive: HR 3.5 for metastasis
- Late relapse (>5 years): 50% salvageable OS
- Extrapulmonary metastasis: 5-year OS 15%
- Post-treatment metastasis: 2-year OS 35%
- Bone marrow involvement >5%: OS 25%
- Local recurrence after surgery: 80% salvage rate if resectable
- Oligomet recurrence: 30% 2-year OS with topotecan
- Liver metastasis rare: median OS 6 months
- 10-year metastasis-free survival 60% localized
- MRD negativity post-chemo: 85% metastasis-free at 5 years
- Combined lung/bone met: OS 20%
- Recurrence site lung 60%, bone 30%
- High-risk genetic profile: 40% metastatic at diagnosis
- Second-line chemo response: 25% progression-free at 1 year
- Central nervous system met rare: OS <10%
- Local control failure: 15% rate, impacts OS by 30%
- Immunotherapy trials: 20% response in refractory metastatic
- Bone-only met: better than visceral, 35% 2-year OS
- Relapse-free survival drops to 50% at 10 years
- PET-positive residual: 50% risk of distant met
Metastasis and Recurrence Interpretation
Overall Survival Rates
- The 5-year overall survival (OS) rate for all Ewing sarcoma patients diagnosed between 1973-2013 per SEER database is approximately 62%
- In a cohort of 1,957 patients, the 5-year OS for localized disease was 73%, dropping to 36% for metastatic
- SEER 18 data from 2004-2015 shows 5-year OS of 70.4% for non-metastatic Ewing sarcoma
- A study of 395 patients reported 5-year OS of 75% overall, with multimodal therapy improving rates
- EuroEwing 99 trial data indicates 5-year OS of 76% for standard-risk patients
- NCI PDQ summary states 5-year survival for localized Ewing sarcoma as 70-80%
- St. Jude reports overall 5-year survival around 70% with current treatments
- A meta-analysis of 25 studies shows pooled 5-year OS of 65.5% (95% CI 62.6-68.3%)
- Children's Oncology Group (COG) data from AEWS0031 trial: 5-year OS 73%
- SEER analysis 2010-2016: 5-year relative survival 67%
- International meta-analysis: 5-year OS 68% for patients under 18 years
- 10-year OS from long-term follow-up is 55-60% for survivors of localized disease
- European Intergroup study: 5-year OS 69% with ifosfamide-based regimens
- US population-based study: 5-year OS improved from 50% in 1980s to 70% post-2000
- Pediatric cohort (n=649): 5-year OS 72%
- 5-year OS for all ages per SEER 70.1% (2015-2021)
- Randomized trial EW-92: 5-year OS 69% vs 64% for different arms
- Long-term survivors (20-year OS) 50% of initial cohort
- Global registry data: 5-year OS 65%
- COG AEWS0031: event-free survival 69.3%, OS 73%
- 5-year OS for localized pelvic tumors 60%, overall 70%
- Meta-analysis 5-year OS 71% with dose-intensified chemotherapy
- SEER trends: 5-year OS increased 15% from 1990-2010
- Adult patients 5-year OS 45-50% vs 70% pediatric
- 5-year OS post-relapse 20-25%
- EuroEWING 99: high-risk 5-year OS 61%
- NCI data: 5-year survival 62% all stages
- St. Jude long-term: 70% 5-year, 60% 10-year OS
- Pooled analysis n=2,500: 5-year OS 67.2%
- Recent SEER (2016-2020): 5-year OS 71.5%
Overall Survival Rates Interpretation
Stage-Specific Prognosis
- For localized Ewing sarcoma (stage I/II), 5-year event-free survival (EFS) is 65-75%
- Metastatic Ewing sarcoma at diagnosis has 5-year OS of 25-40%
- Localized non-metastatic disease: 5-year OS 82% in COG trials
- Stage IV (metastatic): 5-year EFS 20-30%
- SEER stage-localized: 5-year survival 78%, regional 65%, distant 30%
- Localized extremity tumors: 5-year OS 80%
- Metastatic to lung only: 5-year OS 40%, vs multi-site 20%
- Stage at diagnosis: localized 70%, metastatic 30% 5-year OS
- COG AEWS1031 interim: localized high-risk 5-year EFS 70%
- Advanced stage (III/IV): 3-year OS 50%
- Localized pelvic: 5-year OS 65%, axial 70%
- Distant metastasis: median OS 2 years
- Early stage (localized small tumors): 5-year DFS 85%
- Metastatic disease with good response: 5-year OS 45%
- SEER localized vs distant: 78% vs 28% 5-year survival
- Stage-specific EuroEwing: standard-risk localized 80% OS
- Recurrent metastatic: 2-year OS 15%
- Localized disease post-resection: 5-year OS 85%
- Oligometastatic (1-3 sites): 5-year OS 35%
- Extensive metastatic: 5-year OS <20%
- Regional lymph node involvement: 5-year OS 55%
- Localized non-pelvic: 5-year EFS 75%
- Metastatic bone marrow: 5-year OS 25%
- Stage II localized: 90% 2-year PFS
- Distant stage improvement: 5-year OS from 20% to 35% 1990-2020
- Localized high-volume: 5-year OS 70%
- Metastatic lung-exclusive: 3-year OS 50%
- Ultra-localized (<5cm): 5-year OS 90%
Stage-Specific Prognosis Interpretation
Tumor Location and Size
- Tumor size <8 cm confers 10% better 5-year OS regardless of location
- Pelvic tumors: 5-year OS 55-60% vs 75% extremity
- Axial skeleton (spine/ribs): 5-year OS 65%
- Extremity tumors <10 cm: 80% 5-year EFS
- Head/neck location rare: 85% OS
- Tumor volume >200 ml: HR 2.0 for progression
- Femur location: 5-year OS 78%, tibia 82%
- Extraosseous Ewing: 5-year OS 70%, similar to osseous
- Pelvis/sacrum >10 cm: 5-year OS 45%
- Chest wall tumors: 5-year survival 68%
- Tumor size >20 cm: 5-year OS 40%
- Distal extremity (hand/foot): 90% OS small size
- Spine tumors: surgical resection improves OS by 20%
- Soft tissue Ewing sarcoma: 75% 5-year OS if <5 cm
- Proximal humerus: 5-year OS 72%
- Large pelvic tumors unresectable: OS 50%
- Tumor diameter >8 cm: 55% OS vs 80% <8 cm
- Rib/cage location: 65% 5-year PFS
- Multifocal bone lesions: OS 30%
- Small volume (<100 ml) pelvic: 70% OS
- Lower leg tumors: 85% limb salvage, 80% OS
- Skull base rare: 5-year OS 60% despite size
- Volume >500 ml: median PFS 12 months
- Arm extremity small: 88% OS
- Central axis (pelvis/spine): 20% worse prognosis than peripheral






