Pleuropulmonary Blastoma

  • Etiology:
    — Heterozygous germline mutation in DICER1
    — Mesenchymal and epithelial components resembling fetal lung
  • Imaging:
    — Type 1 is cystic
    — Type II is mixed cystic and solid
    — Type III is solid and can be multifocal and large in size with mediastinal shift and pleural effusion and does not invade chest wall
  • DDX:
  • Complications: Spontaneous pneumothorax
  • Treatment:
  • Clinical:
    — Extremely rare
    — Most common primary malignancy of lungs in childhood
    — In less than 2 years old

Radiology Cases of Pleuropulmonary Blastoma

CXR and CT of pleuropulmonary blastoma
CXR AP (above) shows complete opacification of the right hemithorax with mediastinal shift to the left. Axial CT with contrast of the chest shows a large mass filling the entire right hemithorax that is solid peripherally and cystic centrally, causing mediastinal shift to the left.