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

Al-Backer N, Puligandla PS, Su W, Anselmo M, Laberge JM

Division of Pediatric Respirology, The Montreal Children's Hospital, Montreal, Quebec, Canada H3H 1P3.

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.

Published 14 November 2006 in J Pediatr Surg, 41(11): e13-5.
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