Key result
Neoadjuvant chemotherapy with the VDC/IE regimen successfully converted an initially unresectable primary pulmonary Ewing sarcoma into a resectable tumor, enabling complete surgical resection.
Why the study?
Primary pulmonary Ewing sarcoma is extremely rare, especially in children, with very few cases reported in the literature.
Case Report (n=1)
No
Neoadjuvant chemotherapy can facilitate tumor conversion to a resectable state in pediatric primary pulmonary Ewing sarcoma, allowing for successful surgical resection.
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.
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Lin et al. (2026) conducted a case report in Primary pulmonary Ewing sarcoma (n=1). Neoadjuvant chemotherapy (VDC/IE regimen) and surgical resection was evaluated on Tumor conversion and surgical resection. Neoadjuvant chemotherapy with the VDC/IE regimen successfully converted an initially unresectable primary pulmonary Ewing sarcoma into a resectable tumor, enabling complete surgical resection.
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