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, can be multifocal, large in size, pleural effusion, mediastinal shift, lack of chest wall invasion, associated with spontaneous pneumothorax
  • Clinical: most common primary malignancy of lungs in childhood, extremely rare, < 2 years old

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.