Pleuropulmonary blastoma

Pleuropulmonary blastoma (PPB) is a rare, aggressive childhood lung tumor that arises from immature lung tissue. It often occurs in young children and is associated with genetic mutations, especially in the DICER1 gene. PPB can present with symptoms like coughing, chest pain, or breathing difficulty, but sometimes is found incidentally. There are different types, ranging from less aggressive cystic forms to more invasive solid tumors. Early diagnosis and treatment, which may include surgery and chemotherapy, are crucial for improving outcomes. PPB’s rarity and complexity require specialized care by pediatric oncologists and thoracic surgeons.