Pleuropulmonary blastoma PPB is a rare, aggressive childhood cancer originating in the lungs or pleura. It progresses through three types I, II, III with increasing severity and is associated with DICER1 gene mutations. Clinical symptoms often mimic common respiratory infections, leading to misdiagnosis. Accurate diagnosis relies on imaging and histopathological examinations, with CT and PETCT scans providing crucial information. Treatment typically involves a multimodal approach, including surgery, chemotherapy, and sometimes radiotherapy. Early diagnosis and comprehensive management significantly improve survival rates, emphasizing the need for vigilant screening and follow -up, especially given the genetic implications of DICER1 mutations.
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Prakash et al. (2024) studied this question.
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