title: Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?
abstract: BACKGROUND
  The management of congenital cystic lung lesions is controversial. Arguments for routine resection during infancy include the possibility of the lesion being Type I pleuropulmonary blastoma (PPB) rather than a cystic congenital pulmonary airway malformation (CPAM). We aimed to identify clinical and radiological features that might distinguish between CPAM and PPB and to develop a diagnostic algorithm based on these features.
  
  
  METHODS
  All recorded cases of Type I PPB were retrieved from the International PPB Registry and compared with an institutional cohort of children undergoing resection of CPAM (2002-2013) that was noted at some stage to be at least partially cystic. Regression models were created to identify variables that might differentiate CPAM from PPB. Odds ratio (OR) and positive predictive value (PPV) were calculated for each variable and a decision algorithm developed.
  
  
  RESULTS
  In 112 cases of Type I PPB and 103 of CPAM, factors favoring a diagnosis of CPAM included prenatal detection (OR 89.4), systemic feeding vessel (OR 61.7), asymptomatic (OR 8.0), and hyperinflated lung (OR 6.6). Factors favoring a diagnosis of PPB included bilateral or multisegment involvement (OR 2.4). A decision algorithm that helps to identify lesions requiring resection and those which can be safely observed is presented.
  
  
  CONCLUSIONS
  Clinical and radiological features can help to differentiate between CPAM and PPB. Our algorithm allows identification of children at higher risk of PPB in whom we would recommend resection and those at low risk in whom continued close observation is safe.
authors.affiliation: The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Adina
authors.initials: A
authors.lastname: Feinberg
authors.affiliation: Faculty of Medicine, University of Southampton, Southampton, UK.
authors.forename: Nigel J
authors.initials: NJ
authors.lastname: Hall
authors.affiliation: International Pleuropulmonary Blastoma (PPB) Registry, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, USA.
authors.forename: Gretchen M
authors.initials: GM
authors.lastname: Williams
authors.affiliation: International Pleuropulmonary Blastoma (PPB) Registry, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, USA.
authors.forename: Kris Ann P
authors.initials: KA
authors.lastname: Schultz
authors.affiliation: Kaiser Permanente Roseville Women and Children's Center, Roseville, CA, USA.
authors.forename: Doug
authors.initials: D
authors.lastname: Miniati
authors.affiliation: Children's National Medical Centre, Washington, DC, USA.
authors.forename: D Ashley
authors.initials: DA
authors.lastname: Hill
authors.affiliation: Lauren V Ackerman Laboratory of Surgical Pathology, Washington University Medical Centre, St. Louis, MO, USA.
authors.forename: Louis P
authors.initials: LP
authors.lastname: Dehner
authors.affiliation: International Pleuropulmonary Blastoma (PPB) Registry, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, USA.
authors.forename: Yoav H
authors.initials: YH
authors.lastname: Messinger
authors.affiliation: The Hospital for Sick Children, Toronto, ON, Canada. Electronic address: jacob.langer@sickkids.ca.
authors.forename: Jacob C
authors.initials: JC
authors.lastname: Langer
mesh_terms: D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000175:diagnosis*; D003661:Decision Support Techniques; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000151:congenital / Q000175:diagnosis*; D008297:Male; D016017:Odds Ratio; D011237:Predictive Value of Tests; D018202:Pulmonary Blastoma / Q000151:congenital / Q000175:diagnosis*; D012042:Registries; D012189:Retrospective Studies
keywords: Bronchopulmonary sequestration; Congenital cystic adenomatoid malformation; Congenital lung lesion; DICER1; Pleuropulmonary blastoma; Thoracic surgery