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Radiological findings in children with primary mediastinal Ewing sarcoma/primitive neuroectodermal tumors: a
1Department of Radiology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, China.
Insights
Mediastinal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) in children presents with distinct CT findings. This study summarizes key radiological features and clinical characteristics for improved identification of pediatric mediastinal ES/PNET.
Area of Science:
- Pediatric Oncology
- Radiology
- Medical Imaging
Background:
- Mediastinal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare but aggressive malignancy in children.
- Accurate radiological assessment is crucial for diagnosis and treatment planning.
Purpose of the Study:
- To summarize the radiological findings and clinical characteristics of pediatric mediastinal ES/PNET.
- To identify distinctive computed tomography (CT) features aiding in the diagnosis of mediastinal ES/PNET.
Main Methods:
- Retrospective review of clinical and imaging data from 6 pediatric patients with pathologically confirmed primary mediastinal ES/PNET.
- Analysis of tumor size, location, boundaries, internal characteristics (necrosis, calcification, enhancement), and invasion patterns.
Main Results:
- Tumors ranged from 3.8 to 16.0 cm, with unclear boundaries in all cases.
- Common locations included posterior and mid-posterior mediastinum; all cases showed necrosis and cystic degeneration.
- Invasion of adjacent structures (spinal epidural, ribs, vertebral body, chest wall) and pleural effusion were frequent findings.
Conclusions:
- Pediatric mediastinal ES/PNET exhibits characteristic CT findings.
- These distinctive features can facilitate early and accurate diagnosis, guiding subsequent management.
Abstract:
The goal of this study was to summarize the radiological findings and clinical characteristics of mediastinal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) in children. A retrospective review was conducted on the clinical and imaging data of 6 children with primary mediastinal ES/PNET that was confirmed by pathology. There were 3 girls and 3 boys in this study, aged between 2 and 11 years old. The tumors exhibited a range of maximum diameters for each child, spanning from 3.8 to 16.0 cm, and the boundaries of 6 tumors were unclear. Furthermore, the tumors were located in the posterior mediastinum in 2 cases, in the mid-posterior mediastinum in 3 cases, and in the anterior middle posterior mediastinum in 1 case. Necrosis and cystic degeneration were found in all 6 cases, and calcification was found in 2 cases, and the solid part of the tumors showed moderate heterogeneous progressive enhancement. The spinal epidural was involved in 2 cases; adjacent ribs were invaded in 2 cases; the adjacent vertebral body and appendage were invaded in 1 case, and the chest wall was invaded in 4 cases. Finally, pleural effusion occurred in 4 cases, including 1 case with pericardial effusion; 3 patients with pleural metastasis. In conclusion, the computed tomography (CT) findings of primary ES/PNET originating in childhood show a level of distinctiveness that facilitates their identification.
