Purpose: To describe the imaging findings, clinical presentation and follow up of pleuropulmonary blastoma (PPB) in children.
Materials and methods: Authors present a retrospective review of three young children with pathologically proven PPB seen from 1992 to 2006 in a pediatric hospital. The imaging findings on simple views, CT and MRI are presented.
Results: The first patient showed two bilateral well-defined solid lung lesions on chest X-rays, with homogeneous low attenuation on CT. Patient is free of disease following chemotherapy and surgical treatment. The second patient displayed a right tension pneumothorax. After drainage, he presented on chest X-rays and CT an underlying multicystic lesion. Following chemotherapy and surgical treatment, he presented two pulmonary metastases, which were treated with chemotherapy and surgery. The patient is now free of disease. The third patient showed a complete opacification of the left hemithorax due to a massive pleural effusion; a pleuropulmonary solid mass was seen on US, CT and MRI. The disease progressed with mediastinal, orbital and abdominal metastasis. The patient eventually died.
Conclusion: PPB is a rare chest tumor seen in young children that can present with diverse radiological findings, and sometimes can arise in congenital cystic lung lesions. CT is the gold standard technique both for diagnosis and follow-up of these tumors.