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Cutaneous and subcutaneous Ewing's sarcoma: an indolent disease
E Chow1, T E Merchant, A Pappo
1Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA. edward.chow@tsrcc.on.ca
International Journal of Radiation Oncology, Biology, Physics
|February 8, 2000
Summary
Cutaneous and subcutaneous Ewing's sarcoma (ES) shows an indolent course with a favorable prognosis when treated with combined modality therapy. This approach leads to a high rate of local control and low metastasis, suggesting potential for reduced treatment intensity.
Area of Science:
- Oncology
- Surgical Pathology
- Radiation Oncology
Background:
- Extraosseous Ewing's sarcoma (ES) typically affects deep soft tissues.
- Primary cutaneous or subcutaneous ES is rare, with limited data on its clinical behavior.
Purpose of the Study:
- To analyze the outcomes and patterns of failure in patients with cutaneous or subcutaneous ES.
- To evaluate the efficacy of combined modality therapy for superficial ES variants.
Main Methods:
- Retrospective review of 14 patients with cutaneous or subcutaneous ES treated between 1985 and 1997.
- All patients received chemotherapy (vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide, dactinomycin).
- Surgical resection and radiotherapy to the tumor bed were administered based on institutional or cooperative group protocols.
Main Results:
- No patients presented with metastatic disease; all had localized superficial ES.
- Complete surgical resection was achieved in 13 patients, with negative margins in 10.
- Median follow-up was 77 months, with no local recurrence or distant metastasis observed.
Conclusions:
- Cutaneous and subcutaneous ES exhibit an indolent course and favorable prognosis with combined modality treatment.
- High local control and low metastasis rates support exploring less intensive chemotherapy and tailored radiation therapy to minimize toxicity.
- Further trials are investigating the omission of radiation therapy in completely resected cases to maintain high cure rates while reducing treatment-related sequelae.