Pleuropulmonary Blastoma in a Pediatric Patient: A Case Report and Review of Current Diagnostic and Treatment Strategies.

Pleuropulmonary blastoma (PPB) is a rare, highly aggressive pulmonary tumor that typically presents in the pediatric population. The overall prognosis of PPB is poor, although type I PPB can regress spontaneously. In this case report, the authors present a 3.5-year-old boy histopathologically diagnosed with PPB type III, who underwent three courses of the ifosfamide, vincristine, actinomycin D, and doxorubicin (IVADO) chemotherapy regimen after surgical resection. Although the initial post-chemotherapy evaluations showed no residual or metastatic tumor, based on PET-CT scan findings, it relapsed 4 months after the last chemotherapy course. Finally, the patient expired due to disease progression. In addition to the case presentation, the authors provide an overview of PPB and discuss how this case illustrates key diagnostic and management challenges in advanced PPB type III.