Friday, May 17, 2013

Ewing's Family of Tumors (EFTs)

Ewing's is the second most common malignant bone tumor in children, comprising 10-15% of childhood bone cancers. Ewing's tumors have characteristic small round blue cells. The Ewing's family of tumors include:
    • Ewing's tumor of bone, or Ewing's sarcoma of bone
    • Extraosseous Ewing's (tumor outside of the bone)
    • Primitive neuroectodermal tumor (PNET)
    • Askin's tumor (PNET of the chest wall).
Ewing's family of tumors most frequently occurs in teenagers. It can occur in any bone in the body, but it is most common in the pelvis, thigh, lower leg, upper arm, and rib. Dr. James Ewing who described the tumor in the 1920's distinguished the tumor from osteosarcoma on the basis that it was responsive to radiotherapy.

Treatment for Ewing's Family of Tumors

Ewing's family of tumors is treated with multi-agent chemotherapy, radiation therapy, and/or surgical removal of the primary tumor. Chemotherapy can include vincristine, doxorubicin, and cyclophosphamide altering with ifosfamide and etoposide.
It is important that Ewing's patients are evaluated by specialists immediately after diagnosis from all disciplines including radiologists, chemotherapists, pathologists, surgeons or orthopedic oncologists, and radiation oncologists. As in osteosarcoma, a team approach is necessary, especially if limb-sparing procedures are planned.

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