Presentation
Chest pain and dyspnea.
Patient Data
The left hemithorax large extrapulmonary soft tissue mass lesion involves almost the whole left hemithorax. It measures 14.5 x 16.8 x 23 cm along its max TS, AP, and CC dimensions. It shows a heterogenous appearance with internal cystic areas of breakdown. It infiltrates the chest wall anteriorly along its upper aspect. It is associated with sclerosis and periosteal reaction of the anterior aspect of the 4th rib. Multiple scattered basal pleural masses (likely metastatic). Moderate pleural effusion. Left lung collapse and contralateral mediastinal shift are noted.
Case Discussion
Pathologically proven case of Ewing sarcoma of chest wall.
Ewing sarcoma family of tumors (ESFT), also referred as Ewing sarcomas of the chest wall, are malignant tumors affecting children and young adults, originating either from the osseous structures or the soft tissues of the chest wall.
On imaging, they are usually characterized as large extrapulmonary invasive soft tissue masses that are heterogeneous due to the presence of hemorrhage, necrosis, or cystic changes. Enhancement is intense and there is increased uptake in nuclear medicine studies. Calcifications are uncommon and a small pleural effusion is frequently present.
Originally a small round blue cell tumor of the soft tissues of the chest was described and became known as an Askin tumor or peripheral primitive neuroectodermal tumor (pPNET). All three entities are now thought to belong to the same tumor family, now referred to as Ewing sarcoma family of tumors (ESFT) 1-3.
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