Pleuropulmonary blastoma (PPB) is a rare pediatric thoracic malignancy that typically presents with nonspecific respiratory symptoms and only exceptionally with spontaneous hemothorax. We report 3 pediatric cases in which PPB initially manifested as severe spontaneous hemothorax: a 2-year-old boy presenting in hemorrhagic shock with right-sided hemothorax (PPB type III), a 3-year-old boy with nontraumatic left hemothorax (PPB type II), and a 3-year-old girl initially suspected of having a vascular malformation who was ultimately diagnosed with PPB type II. In all cases, prompt recognition and a coordinated multidisciplinary approach involving emergency management, surgery, oncology, and intensive care enabled effective treatment. All patients completed therapy and are alive and in good clinical condition at the last follow-up. These cases highlight that, although uncommon, PPB should be included in the differential diagnosis of spontaneous hemothorax in pediatric patients.
Buconi et al. (Thu,) studied this question.