Authors' objectives:

The aims of this review are:

- To perform the first systematic review of studies of tumour markers in the Ewing's sarcoma family of tumours (ESFT) and in neuroblastomas in order to identify measures of potential clinical value for the clinical areas of screening, diagnosis, prognosis and monitoring; the review focuses particularly on the role of markers for defining prognosis.

- To facilitate the development of future research strategies, including improvement of the standard of scientific reporting and specification of deficiencies in the literature.

Authors' recommendations: There is currently insufficient evidence to judge the clinical role of tumour markers in the treatment of the two childhood malignancies we studied. A large number of markers have been studied in the literature but the majority of studies are so poorly designed and reported that strong clinical conclusions cannot be made from this systematic review. However, we did manage to identify markers that showed possible prognostic importance. For Ewing's sarcoma family of tumours (ESFT), the following were found to be potentially important prognostic tools and associated with a worse outcome: high levels of serum lactate dehydrogenase, lack of S-100 protein expression in the tumour, and lack of expression of the EWS-FLI type 1 fusion transcript in the tumour. For neuroblastomas, the following were found to be potentially important tools and associated with a worse outcome: amplification of the MYC-N gene; expression of diploid cells (a DNA index of 1) in the tumour; high expression of neurone-specific enolase in the tumour at diagnosis; high serum levels of lactate dehydrogenase and/or ferritin; high multidrug resistance gene-product expression in the tumour; gain of chromosome 17q; deletion of chromosome 1p; low tumour expression of CD44 and/or TrkA; and a low urinary VMA:HVA ratio. Clinical interpretation of the above findings is very difficult because of poor and heterogeneous reporting in the literature identified. The benefits of using these prognostic markers in practice needs to be properly studied in large, multicentre studies. The current rapid development of genetic epidemiology may quickly provide new genetic markers and genetic sequences that supersede many of the markers we have identified as important.

Authors' methods: Systematic review

Project Status: Completed

URL for project: http://www.hta.ac.uk/1075

Year Published: 2003

English language abstract: An English language summary is available

Publication Type: Not Assigned

Country: England, United Kingdom

Organisation Name: NIHR Health Technology Assessment programme

Contact Address: NIHR Journals Library, National Institute for Health and Care Research, Evaluation, Trials and Studies Coordinating Centre, Alpha House, University of Southampton Science Park, Southampton SO16 7NS, UK

Contact Name: journals.library@nihr.ac.uk

Contact Email: journals.library@nihr.ac.uk

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