Gastrointestinal polyps are mucosal overgrowths that, if unchecked, can undergo malignant transformation. Although relatively uncommon in the pediatric age group, they can be the harbingers of multiorgan cancer risk and require close management and follow-up. Additionally, as many polyposis syndromes are inherited, appropriate genetic testing and management of relatives is vital for the health of the entire family. In this review, we discuss both common and uncommon childhood gastrointestinal polyposis syndromes in terms of clinical presentation, management, and surveillance. We also detail any additional malignancy risk and surveillance required in the pediatric age group (<21 years old). Through this review, we provide a framework for gastroenterologists to manage the multifaceted nature of pediatric polyposis syndromes.
*Division of Oncology, Department of Pediatrics
†Division of Gastroenterology, Department of Medicine
‡Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia
§Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Address correspondence and reprint requests to Suzanne P. MacFarland, MD, Division of Oncology, The Children's Hospital of Philadelphia, 3501 Civic Center Blvd., Philadelphia, PA 19104-4302 (e-mail: firstname.lastname@example.org).
Received 15 January, 2019
Accepted 4 April, 2019
B.W.K. for Janssen Pharmaceuticals (travel) and Exact Sciences (consulting); no other potential conflicts of interest to declare.
This work is supported by the National Institutes of Health (K12CA076931 SPM, K08 DK099379 BJW, K08DK106489 BWK), the Precious Jules Foundation (SPM) the Audrey E. Evans Endowed Chair in Molecular Oncology (GMB), and The Children's Hospital of Philadelphia Chair's Initiative (GMB).
The authors report no conflicts of interest