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Update on pediatric cancer predisposition syndromes
Joshua D Schiffman1, James I Geller, Erin Mundt
1Center for Children's Cancer Research and Department of Pediatrics, University of Utah, Salt Lake City, Utah 84112, USA. joshua.schiffman@hci.utah.edu
Pediatric Blood & Cancer
|April 30, 2013
Summary
Pediatric cancer predisposition syndromes are increasingly recognized. This review covers new syndromes, genetic testing, and ethical considerations for families with hereditary cancer risk.
Area of Science:
- Pediatric Hematology/Oncology
- Genetics
- Cancer Predisposition Syndromes
Background:
- Hereditary cancer syndromes in pediatric patients are gaining recognition.
- Understanding these syndromes is crucial for pediatric hematologists/oncologists.
- Familial cancer risk impacts entire families, necessitating comprehensive care.
Purpose of the Study:
- To review key topics from the American Society of Pediatric Hematology/Oncology (ASPHO) 2012 workshop.
- To provide an overview of newly described pediatric cancer predisposition syndromes.
- To discuss genetic testing, screening, and ethical issues related to inherited cancer risk in children.
Main Methods:
- Review of presentations from the ASPHO 2012 Annual Meeting.
- Summary of newly identified cancer predisposition syndromes.
- Discussion of genetic testing, screening protocols, and ethical dilemmas.
Main Results:
- Highlights newly described syndromes: Rhabdoid Tumor Predisposition Syndrome, Hereditary Paragangliomas and Pheochromocytoma Syndrome, and DICER1 Syndrome.
- Covers genetic testing and screening strategies for pediatric cancer predisposition.
- Addresses ethical issues like PGD/PGT and incidental findings from whole genome sequencing.
Conclusions:
- Increased awareness and understanding of hereditary cancer syndromes are vital in pediatric oncology.
- Genetic testing and ethical considerations are integral to managing families with inherited cancer risk.
- The review emphasizes the importance of a multidisciplinary approach to pediatric cancer predisposition.
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