Screening Recommendations for Children with Common Cancer Predisposition Syndromes

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The guidelines highlight the genetic nuances associated with childhood cancer predisposition syndromes.
The guidelines highlight the genetic nuances associated with childhood cancer predisposition syndromes.

An estimated 10% of childhood cancers are due to a genetic predisposition. Now, the American Association for Cancer Research (AACR) issued its first consensus screening recommendations for children with common cancer predisposition syndromes.

The recommendations highlight the genetic nuances associated with childhood cancer predisposition syndromes and provide primary and specialty pediatric clinicians with standardized approaches to facilitate appropriate surveillance of children affected by such syndromes.

The Pediatric Cancer Working Group of the AACR convened a workshop that included 65 professionals from 11 countries, including 51 physician directors or co-directors of cancer predisposition programs. The panel reviewed existing data and practices, and established international consensus recommendations for cancer surveillance for the 50 most common syndromes that predispose children to the development of cancer. 

The syndromes were clustered into nine major groups based on the major cancer types with which they are associated: Li-Fraumeni syndrome, neurofibromatoses, overgrowth syndromes and Wilms tumor, neural tumors, GI cancer predisposition, neuroendocrine syndromes, leukemia predisposition, DNA instability syndromes, and miscellaneous syndromes.

The consensus was that a less than 1% prevalence of a particular type of cancer in those aged 20 years or younger is too low and does not warrant surveillance. However, if the prevalence is 5% or greater, screening is warranted. For cancers with prevalence between 1% and 5%, each case must be considered on an individual basis, according to the authors. 

Reference

1. Brodeur GM, Nichols KE, Plon SE, Schiffman JD, Malkin D. Pediatric cancer predisposition and surveillance: an overview, and a tribute to Alfred G. Knudson Jr. Clin Cancer Res. 2017;23(11); e1-e5.
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