Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?

Adina Feinberg; Nigel J Hall; Gretchen M Williams; Kris Ann P Schultz; Doug Miniati; D Ashley Hill; Louis P Dehner; Yoav H Messinger; Jacob C Langer

doi:10.1016/j.jpedsurg.2015.10.019

Can congenital pulmonary airway malformation be distinguished from Type I pleuropulmonary blastoma based on clinical and radiological features?

J Pediatr Surg. 2016 Jan;51(1):33-7. doi: 10.1016/j.jpedsurg.2015.10.019. Epub 2015 Oct 23.

Authors

Adina Feinberg¹, Nigel J Hall², Gretchen M Williams³, Kris Ann P Schultz³, Doug Miniati⁴, D Ashley Hill⁵, Louis P Dehner⁶, Yoav H Messinger³, Jacob C Langer⁷

Affiliations

¹ The Hospital for Sick Children, Toronto, ON, Canada.
² Faculty of Medicine, University of Southampton, Southampton, UK.
³ International Pleuropulmonary Blastoma (PPB) Registry, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN, USA.
⁴ Kaiser Permanente Roseville Women and Children's Center, Roseville, CA, USA.
⁵ Children's National Medical Centre, Washington, DC, USA.
⁶ Lauren V Ackerman Laboratory of Surgical Pathology, Washington University Medical Centre, St. Louis, MO, USA.
⁷ The Hospital for Sick Children, Toronto, ON, Canada. Electronic address: [email protected].

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.

Conclusion: 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.

Keywords: Bronchopulmonary sequestration; Congenital cystic adenomatoid malformation; Congenital lung lesion; DICER1; Pleuropulmonary blastoma; Thoracic surgery.

MeSH terms

Algorithms
Child
Child, Preschool
Cystic Adenomatoid Malformation of Lung, Congenital / diagnosis*
Decision Support Techniques*
Diagnosis, Differential
Female
Humans
Infant
Infant, Newborn
Lung Neoplasms / congenital
Lung Neoplasms / diagnosis*
Male
Odds Ratio
Predictive Value of Tests
Pulmonary Blastoma / congenital
Pulmonary Blastoma / diagnosis*
Registries
Retrospective Studies

Supplementary concepts

Pleuropulmonary blastoma

Grants and funding

R01 CA143167/CA/NCI NIH HHS/United States