Pleuropulmonary blastoma: Long-term survival and literature review

Background and Procedure. Pleuropulmonary blastoma (PPB) identifies differe nt types of pulmonary tumors in the pediatric age. It is extremely uncommon and is known to have a very poor prognosis, with only few cases surviving after 10 years follow-up. Our experience with such a patient and long-term follow-up (12 years) is therefore of interest. She was a 2-year-old girl ad mitted with a history of respiratory distress and underwent a left thoracot omy. The tumor was removed and after 1 month a polychemotherapy course was started using dadinomycin and vincristine for 6 months. The patient remains disease-free 12 years after the operation. Conclusions. The biologic behav ior of the tumor is considered unpredictable, and for this reason much effo rt has been expended in trying to identify prognostic factors. The preopera tive size of the mass (size <5 cm) in our patient, its complete excision, a nd the histologic aspects might be helpful in identifying favorable prognos tic factors. Med. Pediatr. Oncol. 33:372-376, 1999. (C) 1999 Wiley-Liss. In c.