CASE REPORT article
Front. Oncol.
Sec. Pediatric Oncology
Neoadjuvant Chemotherapy-Enabled Tumor Conversion and Surgical Resection in Pediatric Primary Pulmonary Ewing Sarcoma: A Case Report
Provisionally accepted- Lanzhou University Second Hospital, Lanzhou, China
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Background: Ewing sarcoma (EWS) is a highly aggressive malignant tumor that primarily affects the skeletal system in children and adolescents. Primary pulmonary Ewing sarcoma (PPEWS) is extremely rare, particularly in children, with very few cases reported in the literature. Case Presentation: This report describes a 10-year-old girl who was admitted with an intermittent cough lasting over 7 months. Chest computed tomography (CT) revealed a mass in the right lower lung lobe. Bronchoscopic biopsy demonstrated a highly malignant undifferentiated small round cell tumor, with immunohistochemistry confirming EWS (positive for CD99, NKX2.2, and FLI-1). After 8 cycles of VDC/IE neoadjuvant chemotherapy, neoadjuvant therapy enabled tumor conversion from unresectable to resectable status. Postoperative pathology confirmed extraskeletal EWS/peripheral primitive neuroectodermal tumor (pPNET) following right middle and lower lobectomy, with good postoperative recovery. Following a multidisciplinary team (MDT) consensus, the patient initiated adjuvant chemotherapy utilizing the same VDC/IE regimen. As of the latest follow-up, she has successfully completed six cycles of adjuvant chemotherapy, and no clinical or radiological signs of recurrence have been observed. Conclusion: This case underscores the extreme rarity of PPEWS in children and the complexities of its diagnosis and treatment. Neoadjuvant chemotherapy can facilitate tumor conversion, while surgery plays a pivotal role in localized cases with incomplete chemotherapeutic response. Multidisciplinary management is essential for optimizing outcomes.
Keywords: case report, Ewing sarcoma, Lobectomy, Neoadjuvant chemotherapy, pediatric oncology, primary pulmonary tumor
Received: 17 Oct 2025; Accepted: 29 Jan 2026.
Copyright: © 2026 Lin, Li, Li and Yang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Bin Li
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