Systematic review of scapular Ewing's sarcoma and a rare case of massive pleural effusion as primary manifestation.

BACKGROUND: Ewing Sarcoma (ES) ranks second among mesenchymal malignancies in children and young adults. Scapular Ewing sarcoma (SES) represents <4% of ES cases with limited literature. We report a case of SES with uncommon respiratory symptoms in a 37-year-old male and conduct a systematic review to improve comprehension of SES demographics, clinical attributes, and management. LITERATURE REVIEW: A systematic search was conducted in PubMed, Scopus, and Google Scholar including SES case reports and series from 1983 to 2024. Using Covidence, references were screened. Two reviewers independently assessed eligibility, resulting in the inclusion of 32 studies. Risk of bias was assessed using Joanna Briggs Institute (JBI) checklists. A total of 124 SES cases were analyzed. SES predominantly affected adolescents with median age of 16 years; IQR: 12-24, and most commonly presented with shoulder swelling. A case within this cohort exhibited respiratory symptoms. Metastases occurred in 30% of cases, mainly to the lungs and bones. Remission was achieved in 64% of cases, while 29.7% had fatal outcomes. CONCLUSION: This review highlights the predominance of shoulder symptoms and identifies a unique respiratory presentation. Given the high metastatic rate, standardized protocols are needed to ensure oncological control and preserve shoulder function. REGISTRATION: PROSPERO,CRD420251008692.