Pediatric Pleuropulmonary Blastoma Revealed By Massive Hemothorax: Multidisciplinary Diagnostic and Therapeutic
Ilaria Buconi1,2, Angelo Zarfati1,2, Cristina Martucci1
1Surgical Oncology Unit.
Abstract:
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.
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