A systematic review of molecular and biological markers in tumours of the Ewing's sarcoma family

• Published in: European Journal of Cancer Vol. 39; no. 1; pp. 19 - 30
• Main Authors: Riley, R.D, Burchill, S.A, Abrams, K.R, Heney, D, Sutton, A.J, Jones, D.R, Lambert, P.C, Young, B, Wailoo, A.J, Lewis, I.J
• Format: Book Review Journal Article
• Language: English
• Published: Oxford Elsevier Ltd 2003; Elsevier
• Subjects: Biological and medical sciences; Biomarkers, Tumor - analysis; Bone Neoplasms - diagnosis; Bone Neoplasms - genetics; Diseases of the osteoarticular system; Ewing's Sarcoma; Humans; Mass Screening - methods; Medical sciences; Meta-analysis; Oncogene Proteins, Fusion - genetics; Prognosis; Proto-Oncogene Protein c-fli-1; RNA-Binding Protein EWS; Sarcoma, Ewing - diagnosis; Sarcoma, Ewing - genetics; Software Design; Systematic review; Transcription Factors - genetics; Tumors of striated muscle and skeleton; Tumour marker; Systematic review; Prognosis; Tumour marker; Ewing's Sarcoma; Meta-analysis; Human; Ewing sarcoma; Diseases of the osteoarticular system; Biological marker; Genetics; Malignant tumor; Bone; Hybrid gene; Bibliographic review; Metaanalysis
• ISSN: 0959-8049; 1879-0852
• DOI: 10.1016/S0959-8049(02)00500-2

The aims of this study were to perform the first systematic review of molecular and biological tumour markers in tumours of the Ewing's sarcoma family (ESFT), and evaluate the current evidence for their clinical use. A well-defined, reproducible search strategy was used to identify the relevant literature from 1966 to February 2000. Papers were independently assessed for tumour markers used in the screening, diagnosis, prognosis or monitoring of patients with ESFT. Eighty-four papers studying the use of 70 different tumour markers in ESFT's were identified. Low-quality, inconsistent reporting limited meta-analysis to that of prognostic data for 28 markers. Patients with tumours lacking S-100 protein expression have a better overall survival (OS) (hazard ratio (HR)=0.41, 95% confidence interval (CI) 0.19, 0.89) than those with expression; patients with high levels of serum LDH had a worse OS and disease-free survival (DFS) (OS: HR=2.92, CI 2.16, 3.94, DFS: HR=3.38, 95% CI 2.28, 4.99); patients with localised disease and tumours expressing type 1
EWS-FLI1 fusion transcripts had an improved DFS compared with those with other fusion transcript types (HR=0.17, 95% CI 0.079, 0.37). The knowledge base formed should facilitate more informative future research. Improved statistical reporting and large, multicentre prospective studies are advocated.