Ewing Sarcoma is the second most common primary bone
sarcoma with 900 new diagnoses per year in Europe (EU27). It has a poor survival rate in the face of metastatic disease, with no more than 10% survival of the 35% who develop recurrence. Despite the remaining majority having localised disease, approximately 30% still relapse and die despite
salvage therapies. Prognostic factors may identify patients at higher risk that might require differential therapeutic interventions. Aside from phenotypic features, quantitative
biomarkers based on
biological measurements may help identify tumours that are more aggressive. We audited the research which has been done to identify prognostic
biomarkers for
Ewing sarcoma in the past 15 years. We identified 86 articles were identified using defined search criteria. A total of 11,625 patients were reported, although this number reflects reanalysis of several cohorts. For phenotypic markers, independent reports suggest that tumour size > 8 cm and the presence of
metastasis appeared strong predictors of negative outcome. Good histological response (
necrosis > 90%)
after treatment appeared a significant predictor for a positive outcome. However, data proposing
biological biomarkers for practical clinical use remain un-validated with only one secondary report published. Our recommendation is that we can stratify patients according to their stage and using the phenotypic features of
metastases, tumour size and histological response. For
biological biomarkers, we suggest a number of validating studies including markers for 9p21 locus,
heat shock proteins,
telomerase related markers,
interleukins, tumour
necrosis factors,
VEGF pathway, lymphocyte count, and a number of other markers including Ki-67.