Related Experiment Video
Updated: Jun 27, 2026
Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus
Published on: March 6, 2019
[Pleuropulmonary blastoma in children: imaging findings and clinical patterns]
R Mut Pons1, M D Muro Velilla, C Sangüesa Nebot
1Servicio de Radiodiagnóstico Infantil, Hospital Universitario La Fe, Valencia, Spain. raulmut@hotmail.com
Insights
Pleuropulmonary blastoma (PPB) is a rare pediatric chest tumor with varied imaging findings. Early diagnosis and treatment are crucial for managing this rare lung cancer in children.
Area of Science:
- Pediatric Oncology
- Thoracic Imaging
- Rare Tumors
Background:
- Pleuropulmonary blastoma (PPB) is a rare malignant tumor of the lung in children.
- It can arise from congenital cystic lung abnormalities.
- Understanding its diverse presentation is key for timely diagnosis.
Observation:
- This study reviewed three pediatric cases of pathologically confirmed PPB.
- Imaging modalities included X-ray, CT, and MRI.
- Clinical presentations ranged from asymptomatic lung lesions to tension pneumothorax and pleural effusion.
Findings:
- Radiological findings varied significantly, including bilateral solid lesions, multicystic masses, and large pleural effusions with solid components.
- Computed tomography (CT) proved essential for detailed assessment and follow-up.
- Outcomes differed, with two patients achieving disease-free status after multimodal treatment, while one experienced fatal progression.
Implications:
- PPB presents with diverse imaging features, necessitating a high index of suspicion in children with chest abnormalities.
- CT is the gold standard for diagnosing and monitoring PPB.
- Multimodal treatment including chemotherapy and surgery offers potential for cure in some cases.
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.
Related Concept Videos
01:29Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care
01:13Radiological Investigation III: Pulmonary Angiogram and PET Scan
Pulmonary Angiogram
A Pulmonary Angiogram is an invasive procedure involving injecting a contrast medium through a catheter threaded into the pulmonary artery or the right side of the heart to visualize the pulmonary vasculature. Computed Tomography (CT) scans have mainly replaced this...
01:27Pneumothorax-II
Clinical Manifestations:
01:30Radiological Investigation II: MRI and Ventilation Perfusion Scan
Magnetic Resonance Imaging (MRI) and Ventilation Perfusion Scans are two radiological investigations that offer detailed diagnostic images of the body, particularly lung structures.
MRI
MRI uses magnetic fields and radiofrequency signals to distinguish between normal and abnormal tissues. This technology provides a more detailed diagnostic image than CT scans, enabling it to characterize pulmonary nodules, stage bronchogenic carcinoma, and evaluate inflammatory activity in...