Pleuropulmonary blastoma is a rare intrathoracic neoplasm almost solely con
fined to childhood. Survival is poor. The authors report 2 children with ex
tensive intrathoracic disease who are long term survivors after multimodal
therapy. Both children received multiagent neoadjuvant chemotherapy, follow
ed by surgical resection to remove all gross tumor. Postoperative chemother
apy was given to both children; radiotherapy was also given in the second c
ase because of a question of positive tumor margins. Experience supports th
e use of multimodal therapy, including an aggressive surgical approach in t
he potentially curative treatment of this tumor. (C) 2001 by The Society of
Thoracic Surgeons.