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Long-term Outcome of Acral Ewing Sarcoma
Katherine E Mallett1, Mark J Heidenreich1, Zerahiah M Joseph2
1Mayo Clinic, Department of Orthopedic Surgery, Rochester, MN, U.S.A.
Anticancer Research
|February 27, 2022
Summary
Acral Ewing sarcoma, a rare upper extremity bone tumor, was treated with chemotherapy and surgery or radiotherapy. Combined chemotherapy and surgery achieved local control in all patients, offering acceptable functional outcomes.
Area of Science:
- Orthopedic Oncology
- Pediatric Oncology
- Skeletal Tumors
Background:
- Ewing sarcoma typically affects the distal femur or pelvis.
- Acral Ewing sarcoma of the upper extremity is an exceptionally rare presentation.
- Understanding management strategies for rare tumor locations is crucial.
Purpose of the Study:
- To review institutional experience in managing rare acral Ewing sarcomas.
- To evaluate treatment outcomes for this rare tumor subtype.
- To identify optimal therapeutic approaches for upper extremity Ewing sarcomas.
Main Methods:
- Retrospective review of 10 patients with Ewing sarcoma distal to the elbow.
- Analysis of patient demographics, tumor characteristics, and treatment modalities.
- Evaluation of treatment response, local control, metastasis, and survival rates.
Main Results:
- The most frequent site was the metacarpal (4/10 patients).
- Treatment involved chemotherapy with surgery (7/10) or radiotherapy (3/10).
- Combined modality treatment resulted in 5-year survival rates of 55% (metastasis-free) and 60% (disease-specific).
Conclusions:
- Acral Ewing sarcoma is a rare entity requiring specific management considerations.
- Combined chemotherapy and surgical resection provide effective local control.
- This approach yields acceptable functional outcomes for patients with upper extremity Ewing sarcoma.
