PHILADELPHIA -- The American Association for Cancer Research (AACR) has published its first set of consensus screening recommendations for children with common cancer predisposition syndromes in Clinical Cancer Research, a journal of the AACR. These recommendations emerged from the Childhood Cancer Predisposition Workshop held by the AACR Pediatric Cancer Working Group in October 2016.
These recommendations serve to highlight the genetic nuances associated with childhood cancer predisposition syndromes as well as provide primary and specialty pediatric clinicians with standardized approaches to facilitate appropriate surveillance of children affected by such syndromes.
"We are at an evolving time, and in the current era with widespread use of genomic sequencing, several studies indicate that at least 10 percent of childhood cancers arise as a result of genetic predisposition," said chairperson of the Childhood Cancer Predisposition Workshop, Garrett M. Brodeur, MD.
Brodeur, a professor of pediatrics; Audrey E. Evans Chair in Molecular Oncology; associate director of Abramson Cancer Center; and director of the Cancer Predisposition Program at the Children's Hospital of Philadelphia, added, "Given that in children, the overall risk for developing cancer is low, surveillance in the general population is probably not feasible or economically warranted. However, surveillance seems a sound strategy if target populations with an increased risk for developing cancer can be identified."
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 (pediatric oncologists or medical geneticists), seven genetic counselors, three radiologists, three directors of adult cancer predisposition programs, and a pediatric endocrinologist.
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. These syndromes were then 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 percent prevalence of a particular type of cancer in those 20 years of age or younger is too low and, therefore, not worthy of surveillance, and that if the prevalence is 5 percent or more, then we really should screen," Brodeur noted. "Surveillance for cancers with prevalence between 1 and 5 percent would depend on specific factors and be considered on an individual basis."
A major outcome of this workshop is the development of 18 position papers that provide recommendations for surveillance, focusing on when to initiate and/or discontinue specific screening measures, which modalities to use, and how frequent to screen patients.
"We needed to start somewhere for a number of these diseases for which there were no existing protocols. We now have the recommendations for the 50 most common syndromes that predispose children to the development of cancer in their first 20 years of life. The publications will be made freely available online so that anyone in the world--patients, doctors, anybody interested--will be able to read and download them," Brodeur said. "After we gather some experience in following these surveillance protocols, we hope to meet again and combine our experiences to see how they are working and if they should be modified," he added.
An overview of the initiative and the first five position papers, published on June 1, 2017, are freely available to readers online:
- Pediatric Cancer Predisposition and Surveillance: An Overview, and a Tribute to Alfred G. Knudson Jr.
- Pediatric Cancer Predisposition Imaging: Focus on Whole-Body MRI
- Recommendations for Surveillance for Children with Leukemia-Predisposing Conditions
- Recommendations for Childhood Cancer Screening and Surveillance in DNA Repair Disorders
- Clinical Management and Tumor Surveillance Recommendations of Inherited Mismatch Repair Deficiency in Childhood
- Cancer Screening Recommendations for Individuals with Li-Fraumeni Syndrome
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For details regarding the institutions with established centers dedicated to determining and managing hereditary cancer risk that were represented at the AACR Childhood Cancer Predisposition Workshop, go here: http://www.aacr.org/MEMBERSHIP/PAGES/SCIENTIFIC WORKING GROUPS/PEDIATRIC-CANCER-PREDISPOSITION-AND-SURVEILLANCE.ASPX#.WSg1t7czXVh
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About the American Association for Cancer Research
Founded in 1907, the American Association for Cancer Research (AACR) is the world's first and largest professional organization dedicated to advancing cancer research and its mission to prevent and cure cancer. AACR membership includes more than 37,000 laboratory, translational, and clinical researchers; population scientists; other health care professionals; and patient advocates residing in 108 countries. The AACR marshals the full spectrum of expertise of the cancer community to accelerate progress in the prevention, biology, diagnosis, and treatment of cancer by annually convening more than 30 conferences and educational workshops, the largest of which is the AACR Annual Meeting with more than 21,900 attendees. In addition, the AACR publishes eight prestigious, peer-reviewed scientific journals and a magazine for cancer survivors, patients, and their caregivers. The AACR funds meritorious research directly as well as in cooperation with numerous cancer organizations. As the Scientific Partner of Stand Up To Cancer, the AACR provides expert peer review, grants administration, and scientific oversight of team science and individual investigator grants in cancer research that have the potential for near-term patient benefit. The AACR actively communicates with legislators and other policymakers about the value of cancer research and related biomedical science in saving lives from cancer. For more information about the AACR, visit http://www.AACR.org.
About Clinical Cancer Research
Clinical Cancer Research, a journal of clinical and translational research, focuses on innovative clinical research and translational research that bridges the laboratory and the clinic. Clinical Cancer Research presents clinical trials evaluating new treatments for cancer; research on molecular abnormalities that predict incidence, response to therapy, and outcome; and laboratory studies of new drugs and biomarkers that will lead to clinical trials in patients. For more information about Clinical Cancer Research, visit http://clincancerres.aacrjournals.org/ or to submit a manuscript, go to https://ccr.msubmit.net/cgi-bin/main.plex.To interview Garrett Brodeur, contact Julia Gunther at julia.gunther@aacr.org or 215-446-6896.
Journal
Clinical Cancer Research