title: Risk-reduction surgery in pediatric surgical oncology: A perspective.
abstract: OBJECTIVE
  A small percentage of pediatric solid cancers arise as a result of clearly identified inherited predisposition syndromes and nongenetic lesions. Evidence supports preemptive surgery for children with genetic [multiple endocrine neoplasia type 2 (MEN2), familial adenomatous polyposis syndrome (FAP), hereditary nonpolyposis colorectal cancer (HNPCC), and hereditary diffuse gastric cancer (HDGC) and nongenetic [thyroglossal duct cysts (TGDC), congenital pulmonary airway malformations (CPAM), alimentary tract duplication cysts (ATDC), and congenital choledochal cysts (CCC)] developmental anomalies. Our aim was to explore the utility of risk reduction surgery to treat and prevent cancer in children.
  
  
  METHODS
  A systematic review of the available peer-reviewed literature on PubMed was performed using a PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) search strategy, where possible. Search items included "risk reduction surgery", "hereditary cancer predisposition syndrome", "multiple endocrine neoplasia type 2", "familial adenomatous polyposis", "hereditary nonpolyposis colorectal cancer", "hereditary diffuse gastric cancer", "thyroglossal duct cysts", congenital pulmonary airway malformations", "alimentary tract duplication cysts", "malignant transformation", and "guidelines".
  
  
  RESULTS
  We identified 67 articles that met the inclusion criteria describing the indications for prophylactic surgery in surgical oncology. For the genetic predisposition syndromes, 7 studies were related to professional endorsed guidelines, 7 were related to surgery for MEN2, 11 were related to colectomy for FAP, 6 were related to colectomy for HNPCC, and 12 related to gastrectomy for HDGC. Articles for the nongenetic lesions included 5 for techniques related to TGDC resection, 9 for surgery for CPAMs, and 10 for resection of ATDCs. Guidelines and strategies varied significantly especially related to the extent and timing of surgical intervention; the exception was for the timing of thyroidectomy in children with MEN2.
  
  
  CONCLUSIONS
  Current evidence supporting prophylactic surgery in the management of pediatric cancer predisposition syndromes and nongenetic lesions is best delineated for thyroidectomy to prevent medullary thyroid cancer in children with MEN2 (Strength of Recommendation Grade B/C). Despite the lack of pediatric specific evidence-based recommendations regarding the appropriate extent and timing for risk-reduction surgery for FAP, HNPCC, HDGC and nongenetic anomalies, our review represents an opportunity towards understanding the postgenomic development of these lesions and provides current indications and techniques for preemptive cancer prevention surgery in children.
authors.affiliation: Division of Pediatric Surgery,Baptist Children's Hospital,Memphis,TN,United States. Electronic address: John.Sandoval@bmg.md.
authors.forename: John A
authors.initials: JA
authors.lastname: Sandoval
authors.affiliation: Department of Surgery,St. Jude Children's Research Hospital,Memphis,TN,United States.
authors.forename: Israel
authors.initials: I
authors.lastname: Fernandez-Pineda
authors.affiliation: Department of Surgery,St. Jude Children's Research Hospital,Memphis,TN,United States.
authors.forename: Alpin D
authors.initials: AD
authors.lastname: Malkan
mesh_terms: D002648:Child; D006801:Humans; D009386:Neoplastic Syndromes, Hereditary / Q000517:prevention & control / Q000601:surgery*; D010372:Pediatrics; D065286:Prophylactic Surgical Procedures; D000071077:Surgical Oncology; D016896:Treatment Outcome
keywords: Hereditary cancer predisposition syndrome; Nongenetic congenital anomalies; PRISMA; Pediatric solid tumor; Preimplantation genetic diagnosis; Prophylactic cancer surgery; Risk reduction surgery