Ewing's sarcoma medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

The treatment of Ewing's sarcoma depends on the stage of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods. Rather than using the standard staging system, a simpler system is often used when deciding treatment for Ewing's sarcoma. This system divides Ewing's sarcoma of the bone into 2 groups: localized and metastatic.

Medical Therapy

The treatment of Ewing's sarcoma depends on the stage of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods.

Chemotherapy

• Chemotherapy is is given before surgery for Ewing's sarcoma. Additional chemotherapy is usually given 2-3 weeks after surgery.
• The most common chemotherapy combinations used to treat Ewing's sarcoma are:

• Vincristine (Oncovin), doxorubicin (Adriamycin), cyclophosphamide, ifosfamide, etoposide, and mesna
• Vincristine, doxorubicin, cyclophosphamide, dactinomycin (Actinomycin D), etoposide, ifosfamide, and mesna

• Duration of treatment vary depending on location and stage of the disease at diagnosis. Radical chemotherapy may be as short as 6 treatments at 3 week cycles, however most patients will undergo chemotherapy for 6-12 months and radiation therapy for 5-8 weeks.

Treatment

The treatment of Ewing's sarcoma depends on the stage of the tumor. Rather than using the standard staging system, a simpler system is often used when deciding treatment for Ewing's sarcoma. This system divides Ewing's sarcoma of the bone into 2 groups: localized and metastatic. The following are treatment options for all stages of Ewing's sarcoma of the bone:

Localized Ewing's sarcoma

With localized Ewing's sarcoma, the cancer is only in the bone where it started and it has not spread to nearby lymph nodes. Standard treatment options for localized Ewing's sarcoma include the following:

• Chemotherapy
• Local control measures:

• Surgery
• Radiation therapy

Chemotherapy

• Chemotherapy is the primary treatment for localized Ewing's sarcoma.
• Chemotherapy is used because most people who appear to have localized disease have hidden (occult) metastatic disease.
• Chemotherapy is given before surgery or radiation therapy to:

• Treat possible micrometastases cancer cells that have spread from the original (primary) site to other parts of the body in such small amounts that screening or diagnostic tests cannot find them.
• Shrink the tumor.

• It is also given after surgery or radiation therapy to kill all remaining cancer cells.

Radiation

• Radiation therapy may be offered for localized Ewing's sarcoma.
• Radiation is used instead of surgery if the tumor is in a location that makes it inoperable or if surgery would compromise the function.
• It may be given after surgery if the margins contain cancer cells or the surgeon could not remove a wide margin around the tumor.

Surgery

• Surgery may be offered for localized Ewing's sarcoma following chemotherapy.
• The types of surgery that may be done include:

• Limb-sparing surgery
• Amputation
• ReconstructionPotential

• Benefits of surgery include the following:

• If a very young child has Ewing's sarcoma, surgery may be a less-morbid therapy than radiation therapy because of the retardation of bone growth caused by radiation.
• Another potential benefit for surgical resection of the primary tumor is related to the amount of necrosis in the resected tumor. Patients with residual viable tumor in the resected specimen have a worse outcome than those with complete necrosis.

Metastatic Ewing's sarcoma

Metastatic Ewing's sarcoma has already spread to distant parts of the body at the time of diagnosis. It usually spreads to the lungs, other bones, or the bone marrow. Standard treatment options for metastatic Ewing's sarcoma include the following:

• Chemotherapy
• Surgery
• Radiation therapy

Chemotherapy

• Chemotherapy is usually the first treatment for metastatic Ewing's sarcoma.
• Standard treatment for patients with metastatic Ewing's sarcoma utilizing alternating vincristine, doxorubicin, cyclophosphamide, and ifosfamide/etoposide combined with adequate local control measures applied to both primary and metastatic sites often results in complete or partial responses.

Surgery

• Surgery may be used to remove any visible Ewing's sarcoma.

Radiation

• Radiation therapy may be given to the primary tumor and to the metastases.
• The radiation dose depends on the metastatic site of disease:

• Bone and soft tissue: stereotactic body radiation therapy has been used to treat metastatic sites in bone and soft tissue. The median total curative stereotactic body radiation therapy dose delivered was 40 Gy in five fractions (range, 30-60 Gy in 3-10 fractions). The median total palliative stereotactic body radiation therapy dose delivered was 40 Gy in five fractions (range, 16-50 Gy in 1-10 fractions). These short-course regimens with large-dose fractions are biologically equivalent to higher doses delivered with smaller-dose fractions given over longer treatment courses.
• Pulmonary: for all patients with pulmonary metastases, whole lung irradiation should be considered, even if complete resolution of overt pulmonary metastatic disease has been achieved with chemotherapy. Radiation doses are modulated based on the amount of lung to be irradiated and on pulmonary function. Doses between 12 Gy and 15 Gy are generally used if whole lungs are treated.

References

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