Pleuropulmonary Blastoma in Pediatric Lung Lesions

Pediatrics. 2021 Apr;147(4):e2020028357. doi: 10.1542/peds.2020-028357. Epub 2021 Mar 24.


Background: Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy.

Methods: A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB).

Results: None of the 344 prenatally diagnosed lesions had malignant pathology (P < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, n = 11; other PPB, n = 3; adenocarcinoma, n = 1) at a median age of 20.7 months (interquartile range, 7.9-58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel (P = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2-58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43-340.3; P < .0001] and 42.03 [95% CI, 3.51-995.6; P = .0041], respectively).

Conclusions: In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology.

MeSH terms

  • Child, Preschool
  • Cohort Studies
  • DEAD-box RNA Helicases / genetics
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Length of Stay
  • Lung Neoplasms / diagnostic imaging
  • Lung Neoplasms / genetics
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / surgery
  • Mutation
  • Neoplasm Metastasis / genetics
  • Pregnancy
  • Prenatal Diagnosis
  • Pulmonary Blastoma / diagnostic imaging
  • Pulmonary Blastoma / genetics
  • Pulmonary Blastoma / pathology*
  • Pulmonary Blastoma / surgery
  • Respiratory Distress Syndrome, Newborn / etiology
  • Retrospective Studies
  • Ribonuclease III / genetics
  • Tomography, X-Ray Computed


  • DICER1 protein, human
  • Ribonuclease III
  • DEAD-box RNA Helicases

Supplementary concepts

  • Pleuropulmonary blastoma