What are Ewing sarcoma spinal tumors?

Updated: Nov 01, 2018
  • Author: Andrew A Sama, MD; Chief Editor: Jeffrey A Goldstein, MD  more...
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Answer

Ewing sarcoma is the most common nonlymphoproliferative primary malignant tumor of the spine in children. Lesions of the spine make up 3-10% of all primary sites of Ewing sarcoma. Metastatic foci of Ewing sarcoma involving the spine are more common than primary lesions of the spine. Patients with Ewing sarcoma usually present between the ages of 10 and 20 years.

The most common site of occurrence in the spine is the sacrococcygeal region, followed by the lumbar and thoracic segments. Ewing sarcoma rarely occurs in the cervical spine. Lesions are centered primarily in the vertebral body but they can extend into the posterior elements. [27]

Plain radiographs reveal permeative bone lysis, osseous expansion, or sclerosis. Diffuse sclerosis is observed in 69% of spinal lesions and is associated with osteonecrosis. CT and MRI demonstrate osseous involvement as well as surrounding soft tissue involvement. However, MRI is nonspecific.

Tissue from a Ewing sarcoma is composed of sheets of small, round, blue cells divided by septa, scant cytoplasm, and abundant collagen. Areas of osteonecrosis are found in spinal lesions. These correspond to the sclerotic areas observed on plain x-rays, as discussed above. Genetically, patients with Ewing sarcoma are found to have an 11;12 chromosomal translocation.

Before the advent of chemotherapy, the survival rate for patients with Ewing sarcoma was dismal because of the inability to completely resect these lesions, especially in the axial skeleton.

Radiation and chemotherapy are the current mainstays of treatment of Ewing sarcoma in the spine, achieving almost 100% local control with an 86% long-term survival rate in patients with spinal Ewing nonsacral sarcomas. Sacral tumors have a 62% local control rate and only a 25% long-term survival rate because of the tendency for delayed clinical presentation and larger tumor size. The most important prognostic indicator for survival of Ewing sarcoma is the tumor's response to chemotherapy.


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