Rare origin - Ewing's sarcoma of the pleura: a case report and literature review

Abstract

Ewing sarcoma (ES) was first reported by Ewing in 1921. It is the second largest malignant bone tumor in children and adolescents, typically occurring in the bones of trunk or limbs . Extraskeletal Ewing sarcoma (EES) was first reported by Tefft et al. in 1969 and is extremely rare, accounting for less than 1% of all sarcomas. It can occur in any part of soft tissue, mostly in the trunk and lower limbs, and rarely in the pleura. We report a 22-year-old case of extraosseous Ewing sarcoma of pleural origin discovered and pathologically confirmed by physical examination. We report its CT manifestations and pathological results, and review the literature to summarize and analyze the clinical and imaging characteristics of extraosseous Ewing sarcoma, in order to improve our understanding of the disease.

Figure 1

Cross-sectional CT scan of the chest. Figures A and B show the lung window, while C and D show the plain and enhanced images of the mediastinal window. They show a soft tissue mass in the posterior basal segment of the right lower lobe of the lung, under the pleura and adjacent to the spinal column, with a smooth edge that is close to the broad base of the pleura, unclear boundary, heterogeneous contrast enhancement, and stronger periphery than the center.

Figure 2

PET-CT scan appearance: Cross section CT plain scan (Figure A) shows a pleural mass in the right posterior inferior rib, PET (Figure B) shows a high uptake area of 18F-FDG, and PET-CT (Figures C, D) fusion shows a pleural hypermetabolic mass invading the right 11th posterior rib.