Extraosseous Ewing’s sarcomas (EES) are extremely rare neuroectodermal tumors, and Hammer et al had reported the first case in 1989(3). In our case, the child was four years old. But the usual age scope of the preponderance of the published cases was in the second decade of life, indicating a possible early presentation or delayed diagnosis in our study (1).
Although the diagnosis of EES primarily based on histology, also clinical and radiological features had a significant role in the diagnose of EES and differential diagnosis from other sarcomas (4). An especially CT scan is useful in describing the extent of the tumor and confirm that the soft tissue mass is completely extraosseous. In the present case, an enhanced chest CT-scan had performed that revealed heterogeneously enhanced mass with multiple cystic necrosis hypodense areas involving the whole left hemithorax associated with compression of the mediastinum to the right side. No calcification had detected within the tumor. Also, we have done a whole-body CT scan, and no metastasis had demonstrated. Extraosseous Ewing's Sarcoma is an aggressive tumor having local recurrences and metastases but, the treatment of choice is an early surgical intervention with intensive chemotherapy and radiation therapy to eliminate any residual microscopic disease (5). Even though our patient had an aggressive character of the tumor, fortunately, no metastasis had detected.
In conclusion, primary extraosseous Ewing's sarcoma is an extremely rare soft tissue neuroectodermal tumor that is histologically imperceptible from the other osseous form. Due to its rarity and aggressive character, it has no particular guidelines for the management of this disease and needed combined modality treatment. Also, it should be considered in the differential diagnosis of children and young adults presenting with primary pulmonary mass.