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Pleuropulmonary blastoma (PPB) identifies different 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 admitted with a history of respiratory distress and underwent a left thoracotomy. The tumor was removed and after 1 month a polychemotherapy course was started using dactinomycin and vincristine for 6 months. The patient remains disease‐free 12 years after the operation.

The biologic behavior of the tumor is considered unpredictable, and for this reason much effort has been expended in trying to identify prognostic factors. The preoperative size of the mass (size <5 cm) in our patient, its complete excision, and the histologic …