Patient-Reported Functional and Quality of Life Outcomes in a Large Cohort of Long-Term Survivors of Ewing Sarcoma

Background: Little data exist regarding long-term functional and quality of life (QOL) outcomes for survivors of Ewing sarcoma (ES). Specifically, there are few reports assessing the impact of patient characteristics and local therapy modalities on patient-reported outcomes (PRO). Procedure: Long-term survivors of ES treated between 1977 and 2009 completed self-assessed functional and QOL surveys, using the Toronto Extremity Salvage Score (TESS) and PEDSQL 4.0 generic core instruments, respectively. Statistical analyses were performed to correlate these outcomes to clinical presentations and treatment regimens. Results: Subjects (n=74) with a mean duration from diagnosis of 17.8 years completed the survey. Mean TESS and PEDSQL total scores for the entire cohort were 93.0±10.3 and 81.6±18.0, respectively. No significant differences were appreciated between adult and pediatric patients, although increasing age was associated with lower functional scores (P=0.04). Mean PEDSQL total scores were significantly lower for female patients (74.6±19.7) compared to males (85.7±15.7) (P=0.01). Mild to moderate disability was reported by 32% of respondents and was associated with lower TESS and PEDSQL scores. Local control modality did not significantly affect functional and QOL scores. Patients with pelvic tumors had significantly lower TESS scores compared to those with nonpelvic tumors (P=0.04), especially amongst patients treated with both surgery and radiation. Conclusions: Although many survivors of ES report excellent functional and QOL outcomes, a significant number report long-term disability and impairments. Older patients, females, and those with pelvic primary tumors appear at the greatest risk for long-term decrements and may benefit from early therapeutic interventions.