CHOP Oncologist Chairs AACR Pediatric Cancer Predisposition Workshop

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International Update

Predisposition for cancer in children was once considered rare, except for a very few cancers such as hereditary retinoblastoma. However, with the increasing use of genomic sequencing, the understanding of predisposition is rapidly evolving: Now at least 10% of childhood cancers are considered a result of genetic predisposition, and it is probably much higher.

Garrett Brodeur, MD Garrett Brodeur, MD In response to this knowledge, in October 2016 the American Association for Cancer Research Pediatric Cancer Working Group convened the Pediatric Cancer Predisposition and Surveillance Workshop, chaired by Garrett M. Brodeur, MD, Director of the Cancer Predisposition Program at Children’s Hospital of Philadelphia. The aim of the workshop was to develop recommendations on cancer surveillance — tests done to detect cancers — so they can be detected at an earlier stage. The recommended surveillance would be different depending on the types of cancer to which an individual was predisposed.

There are several challenges to identifying children who are predisposed. “One is that individual syndromes that can be surveilled for cancer predisposition are rare,” says Brodeur. “At this time we only have established protocols or recommendations for cancer surveillance for a few disorders, but such protocols do not exist for most others.”

Children with cancer predisposition can be identified in several ways, such as family history of the same or similar type of cancer, bilateral or multiple cancers in the same individual, or sometimes based on physical findings that indicate an individual has a certain predisposition syndrome (eg, café-au-lait spots indicate possible neurofibromatosis).

The workshop members addressed several practical questions: “If surveillance for a particular type of cancer is recommended,” Brodeur explains, “what should you do, and how often do you do it? When do you start screening — at birth, or when they are a certain age? When do you stop screening, if ever? Does the screening protocol change over time?”

The workshop created recommendations for the 50 most common syndromes that predispose children to cancer in their first 20 years of life. An overview of the initiative and 16 position papers (5 published on June 1, 2017, and 11 published on July 1, 2017) are freely available online at AACR’s “Clinical Cancer Research.” On June 1, AACR’s blog, “Cancer Research Catalyst,” published a Q&A with Brodeur.