Multimodal management of extra skeletal Ewing sarcoma: A case series and clinical insights from a dedicated orthopedic oncology team
Introduction: Extra-skeletal Ewing sarcoma (EES) is a rare and aggressive soft tissue malignancy, distinct from osseous Ewing sarcoma. With an incidence of approximately one case per 2.5 million individuals, EES is one of the least encountered tumors in clinical practice. It presents challenges due...
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| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2025-04-01
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| Series: | Journal of Orthopaedic Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2773157X25000190 |
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| Summary: | Introduction: Extra-skeletal Ewing sarcoma (EES) is a rare and aggressive soft tissue malignancy, distinct from osseous Ewing sarcoma. With an incidence of approximately one case per 2.5 million individuals, EES is one of the least encountered tumors in clinical practice. It presents challenges due to its rapid progression, early metastasis, and poor prognosis. Treatment strategies often rely on extrapolation from bone Ewing sarcoma protocols, though their effectiveness in EES remains uncertain. This case series aims to review the clinical presentation, treatment, and outcomes of four EES patients. Methods: This retrospective case series analyzed four patients diagnosed with EES. Demographic data, tumor characteristics, treatment modalities (surgery, chemotherapy, and radiotherapy), and survival outcomes were collected. Three patients underwent surgery with adjuvant therapies, and one patient underwent radiotherapy, using standard Ewing sarcoma chemotherapy regimens. Kaplan-Meier survival curves were utilized to assess overall survival (OS) and disease-free survival (DFS). Results: Four patients (ages 15–29) were included.•Patient 1 (pelvic EES): DFS 7 months, OS 13 months.•Patient 2 (knee EES): DFS and OS 74 months.•Patient 3 (hip EES): DFS and OS 35 months.•Patient 4 (presacral EES): Palliative care, survived 25 months.The 12-month OS probability was 75 %, and DFS at 60 months was 66.6 %. Conclusion: EES is a highly aggressive malignancy, yet multimodal treatments combining surgery, chemotherapy, and radiotherapy show promise in extending survival. Even palliative surgery can provide meaningful survival benefits. Further research is needed to optimize treatment protocols for this rare tumor. |
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| ISSN: | 2773-157X |