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Recognizing and Managing Children with a Pediatric Cancer Predisposition Syndrome: A Guide for the Pediatrician
Insights
Pediatricians play a crucial role in identifying inherited cancer predisposition syndromes in children. Early recognition of warning signs and family history red flags aids in timely referrals for genetic evaluation and management.
Area of Science:
- Pediatric Oncology
- Clinical Genetics
- Cancer Predisposition Syndromes
Background:
- An estimated 8-10% of pediatric cancer cases are linked to inherited cancer predisposition syndromes.
- Pediatricians are essential in the early identification and management of these syndromes.
- Key roles include recognizing malignancy symptoms, assessing family history for hereditary cancer risks, and providing primary care for affected children.
Purpose of the Study:
- To equip pediatricians with tools to identify children at risk for inherited cancer syndromes.
- To review clinical warning signs of childhood malignancy and hereditary cancer 'red flags'.
- To provide guidance on cancer genetics referrals and management of pediatric cancer syndromes.
Main Methods:
- This is a review article synthesizing current knowledge on pediatric cancer predisposition.
- It compiles clinical signs, personal and family history indicators, and genetic evaluation criteria.
- The review details major pediatric solid tumor syndromes and surveillance recommendations.
Main Results:
- Provides a comprehensive list of clinical warning signs for childhood cancers.
- Outlines family history 'red flags' indicative of hereditary cancer.
- Offers checklists to facilitate cancer genetics evaluation referrals.
Conclusions:
- Pediatricians can effectively identify and refer children with potential inherited cancer syndromes.
- Understanding key features of pediatric cancer syndromes and surveillance guidelines is crucial.
- This review serves as a practical resource for pediatricians in managing at-risk children.
Abstract:
It is estimated that at least 8% to 10% of children diagnosed with cancer have an inherited cancer predisposition syndrome. Pediatricians may be called upon to (1) identify children with symptoms suggestive of cancer that require further diagnostic testing, (2) identify children who should be referred to cancer genetics based on their personal and family histories, and (3) provide primary care to children who have an inherited cancer syndrome. This review article provides a list of clinical warning signs suggestive of childhood malignancy, discusses the personal and family history "red flags" suggestive of hereditary cancer, offers checklists to help identify patients who are candidates for cancer genetics evaluation, and describes features of the major pediatric cancer syndromes involving solid tumors and surveillance guidelines. This review aims to provide the pediatrician with the tools needed to recognize, refer, and help manage children at risk for pediatric cancer syndromes. [Pediatr Ann. 2018;47(5):e204-e216.].
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