Type 1 pleuropulmonary blastoma in a 3-year-old male with a cystic lung mass.

Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignant tumor that can be associated with cystic lung lesions in children. This neoplasm is histologically characterized by primitive blastoma and a malignant mesenchymal stroma. The authors describe a 3-year-old boy who presented with a history of fever and cough. Radiological imaging demonstrated a large cystic lesion replacing the left lower lobe. The patient underwent thoracoscopic resection of the lesion. Interestingly, the histopathology demonstrated a type 1 PPB. Type 1 lesions are usually observed in young infants, whereas older infants and children tend to present with type 2 or 3 PPB, which carry a poorer prognosis and higher risk of recurrence. Thus, the presence of large or peripherally based lung cysts should raise the suspicion of PPB. Resection is warranted for all such lesions.