Pleuropulmonary blastoma in a child presenting with spontaneous pneumothorax.

ABSTRACT

The authors described a 27-month-old boy with the diagnosis of pleuropulmonary blastoma who presented with spontaneous pneumothorax. The child was admitted to our hospital with the chief complaint of respiratory distress for 8 months. Initial chest X-ray revealed tension pneumothorax on the right side. After chest tube insertion to the right side, a repeated chest X-ray showed minimal pleural effusion and a mass-like lesion at the right lower lung field. Computed tomography (CT) of the chest showed a cavity with intramural mass confined in the right lower lung accompanied with hydropneumothorax. The surgery revealed a cystic and solid mass occupying the right pleural space medially displacing the right lower lung. Total removal of the mass was performed, the histopathologic findings revealed a mixed cystic and solid type of pleuropulmonary blastoma which was composed of primitive blastema with multidirectional differentiation. Combination chemotherapy consisting of vincristine, cyclophosphamide, doxorubicin and dactinomycin was administered two weeks after surgery. The child has been well for almost 6 months since the surgery, without any signs of metastasis or recurrence.