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Is There a Predisposition Gene for Ewing's Sarcoma?
R L Randall1, S L Lessnick, K B Jones
1Sarcoma Services, Department of Orthopaedics, Huntsman Cancer Institute and Primary, Children's Medical Center, The University of Utah, Utah, UT 84112, USA.
Journal of Oncology
|March 20, 2010
Summary
Ewing's sarcoma, a rare childhood cancer, is primarily linked to EWSR1 gene fusions. Research is exploring potential genetic predispositions, as familial cases are currently unreported, suggesting a need for further investigation into hereditary factors.
Area of Science:
- Oncology
- Molecular Biology
- Genetics
Background:
- Ewing's sarcoma is a highly malignant bone and soft tissue tumor affecting children and young adults.
- Most cases exhibit EWSR1 gene fusions with ETS family genes, but other genetic alterations are inconsistently identified.
- The potential for a heritable predisposition to Ewing's sarcoma remains largely unexplored.
Purpose of the Study:
- To review the current evidence regarding hereditary factors in Ewing's sarcoma development.
- To investigate the possibility of undiscovered recurrent genetic abnormalities in Ewing's sarcoma.
- To assess the potential for identifying susceptibility loci through familial case studies.
Main Methods:
- Review of existing literature on Ewing's sarcoma genetics and familial cancer syndromes.
- Analysis of reported cases, including sibling pairs and secondary malignancies.
- Discussion of traditional genetic mapping techniques for identifying susceptibility loci.
Main Results:
- EWSR1 gene fusions are the most consistent molecular alteration found in Ewing's sarcoma.
- Cooperative genetic events like TP53 mutations or CDKN2A deletions are infrequent.
- No significant associations with classic tumor susceptibility syndromes have been identified, and familial cases are unreported.
Conclusions:
- While EWSR1 gene fusions are central to Ewing's sarcoma, other contributing genetic factors, potentially heritable, require further investigation.
- The absence of reported familial cases and links to known syndromes highlights a gap in understanding Ewing's sarcoma etiology.
- Future research should focus on identifying novel genetic alterations and exploring potential hereditary components.
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