Bone and Soft Tissue Sarcomas

Prognostic factors EWS

• Extent of disease
• Metastatic disease unfavorable
• Size of disease ???
• Primary site
• Pelvis least favorable
• Distal bones and ribs most favorable
• Age
• Younger (<10) more favorable
• Histologic ???
• Response to chemotherapy
• Neural differentiation

Treatment EWS

• Multidisciplinary approach must provide both local control and systemic therapy
• Local control measures should not compromise systemic therapy
• When treatment fails, it is usually due to the development of distant metastatic disease

Treatment: Multimodal EWS

• Surgery
• local control where possible
• Radiation
• local control where surgery not possible or incomplete
• Chemotherapy
• control of micrometastases

Treatment: Local Control EWS

• Surgery and/or Radiation therapy
• No randomized studies comparing surgery to radiation therapy
• slightly more local recurrence when radiation used for local control
• current suggestion for surgery where possible without loss of function and without mutilation
• Combination therapy if incomplete resection
• Radiation doses usually 4500 – 5500 cGy

Surgical Indications EWS

• Expendable bone (fibula, rib, clavicle)
• Bone defect able to be reconstructed with modest loss of function
• May consider amputation if considerable growth remaining
• Trend toward improved outcomes with chemo + surgery vs. XRT

Radiation therapy Indications EWS

• Unresectable without significant morbidity
• Pelvic lesions
• Spine lesions
• Lung metastases
• May consider chemo + XRT to allow for surgical resection or add XRT if surgical margins positive

Treatment: Chemotherapy EWS

• All patients require chemotherapy
• Active agents include
• Vincristine, cyclophosphamide, adriamycin, dactinomycin, ifosfamide, etoposide, topotecan, melphalan
• Effective chemotherapy has improved local control rates achieved with radiation to 85-90%
• Role of SCT for high risk Ewing sarcoma still under investigation