EC Clinical and Medical Case Reports

Background: Ewing sarcoma is a rare, aggressive tumor of bone or soft tissue, frequently involving extremities, pelvis, or thorax. Primary extraosseous Ewing sarcoma of the pleura is particularly uncommon and often presents a diagnostic challenge.

Objective: This case report emphasizes pleural effusion as the initial manifestation of primary pleural Ewing sarcoma in a pediatric patient, underscoring that pleurisy should not be assumed to be synonymous with tuberculous pleuritis, even in regions with high tuberculosis prevalence.

Case Presentation: A 4-year-6-month-old boy from a tuberculosis-endemic area, with known tuberculosis contact, presented with fatigue, weight loss, and fever. Clinical and imaging assessments revealed a massive pleural effusion and right thoracic mass. Initial diagnostic workup suggested tuberculosis, with elevated adenosine deaminase (ADA) levels and a positive QuantiFERON test, but pleural fluid cytology and histopathology were negative for malignancy. Further evaluation, including biopsy, confirmed pleural Ewing sarcoma. The patient began chemotherapy, but disease progression led to his death.

Discussion: In endemic areas, pleurisy in children is often attributed to tuberculosis; however, atypical presentations of malignancies like Ewing sarcoma must be considered. Imaging and histopathology are critical for differential diagnosis, as Primary extraosseous Ewing sarcoma of the pleura presents similarly to other thoracic conditions but carries a poorer prognosis. Our case illustrates the importance of considering rare malignancies in differential diagnoses to avoid delays in accurate diagnosis and treatment.

Conclusion: This case emphasizes that pleural effusion in pediatric patients warrants comprehensive evaluation, as it may mask serious underlying conditions like Ewing sarcoma, distinct from tuberculosis despite similar initial presentations.

 Keywords: Ewing Sarcoma; Pediatric Pleural Effusion; Tuberculosis Misdiagnosis; Primary Pleural Tumor

1. Merrouche B and Mahfouf L. “Le sarcome d’Ewing: à propos de 5 cas et d’une revue de la littérature”. Neurochirurgie 3 (2018): 242-243.
2. C Karatziou., et al. “A case of extraskeletal Ewing sarcoma originating from the visceral pleura”. Hippokratia 4 (2011): 363-365.
3. AP Javalgi, et al. “Blue cell tumour at unusual site: retropritoneal Ewings sarcoma”. Journal of Clinical and Diagnostic Research 4 (2016): ED19-ED20.
4. JP Gurria and R Dasgupta. “Rhabdomyosarcoma and extraosseous Ewing sarcoma”. Children12 (2018): 165.
5. Xuexue Zou., et al. “A primary Ewing’s sarcoma of pleura: Case report and literature review”. Respiratory Medicine Case Reports 34 (2021): 101516.
6. SS Murthy., et al. “Ewing sarcoma with emphasis on extraskeletal Ewing sarcoma: a decade’s experience from a single centre in India”. Clinical Pathology 13 (2020): 2632010X20970210.
7. G Wolf., et al. “Massive pleural effusion with dyspnea in a 17-year-old boy as the first sign of Ewing sarcoma”. Journal of Pediatric Hematology/Oncology 5 (2002): 420.
8. C Ozge., et al. “Massive pleural effusion in an 18-year-old girl with Ewing sarcoma”. Canadian Respiratory Journal 5 (2004): 363-365.
9. RAS Kushwaha., et al. “Ewing’s sarcoma presenting as pleural effusion”. Oman Medical Journal 5 (2011): 362-364.
10. D Mathew., et al. “Extra-skeletal Ewing Sarcoma of the chest wall in a child”. SA Journal of Radiology 1 (2019): 1733.