Treatment Option Overview for Ewing Sarcoma

It is important that patients be evaluated by specialists from the appropriate disciplines (e.g., medical oncologists, surgical or orthopedic oncologists, and radiation oncologists) as early as possible. Multidisciplinary review with radiologists and pathologists is often performed at sarcoma specialty centers.

Appropriate imaging studies of the suspected primary site are obtained before biopsy. To ensure that the biopsy incision is placed in an acceptable location, the surgical or orthopedic oncologist (who will perform the definitive surgery) is consulted on biopsy-incision placement. This is especially important if it is thought that the lesion can subsequently be totally excised after initial systemic therapy or if a limb salvage procedure may be attempted. It is almost never appropriate to attempt a primary resection of known Ewing sarcoma at initial diagnosis. With rare exceptions, Ewing sarcoma is sensitive to chemotherapy and will respond to initial systemic therapy. This therapy reduces the risk of tumor spread to surrounding tissues and makes ultimate surgery easier and safer. Biopsy should be from soft tissue as often as possible to avoid increasing the risk of fracture.[1] If the initial biopsy sample is obtained from bone, reserving some tissue without decalcification is required because decalcification denatures DNA and makes genomic profiling of tumor tissue impossible.[2] The pathologist is consulted before biopsy/surgery to ensure that the incision will not compromise the radiation port and that multiple types of adequate tissue samples are obtained. It is important to obtain fresh tissue, whenever possible, for cytogenetics and molecular pathology. A second option is to perform a needle biopsy, as long as adequate tissue is obtained for molecular studies.[3]

Table 5 describes the treatment options for localized, metastatic, and recurrent Ewing sarcoma.

The successful treatment of patients with Ewing sarcoma requires systemic chemotherapy [4-10] in conjunction with surgery and/or radiation therapy for local tumor control.[11-15] In general, patients receive chemotherapy before instituting local-control measures. In patients who undergo surgery, surgical margins and histological response are considered in planning postoperative therapy. Patients with metastatic disease often have a good initial response to preoperative chemotherapy, but in most cases, the disease is only partially controlled or recurs.[16-21] Patients with lung as the only metastatic site have a better prognosis than do patients with metastases to bone and/or bone marrow. Adequate local control for metastatic sites, particularly bone metastases, may be an important consideration.[22]