Discussion Author: Bradley Mathers
Ewingâ€™s sarcoma constitutes 10-15% of all bone sarcomas and is the second most common primary bone malignancy in children and adolescents, with a peak incidence during the second decade of life. This disease is associated with a translocation of the long arms of chromosomes 11 and 22, t(11;22). Although any bone in the body can be involved, Ewingâ€™s sarcoma most often arises in the long bones of the extremities (predominately the femur), and the bones the of the pelvis. Patients may present with localized pain or swelling of a few weeks duration. The pain may be aggravated by exercise and worse at night. A soft tissue mass tender to palpation can sometimes be present. Constitutional symptoms are present in 10-20% of patients. The differential diagnosis should include other common solid tumors of childhood, such as osteosarcoma, primary lymphoma of the bone, spindle cell sarcoma, acute leukemia, and metastases. The diagnosis is confirmed by biopsy.
The radiographic appearance is often described as a â€śpermeativeâ€ť lytic lesion due to the multiple finely destructive lesions that become confluent over time. The cortex is often expanded and the periosteum displaced, causing the sign of Codmanâ€™s triangle. An â€śonion peelâ€ť periosteal reaction, resulting from layers of reactive bone, is characteristic of Ewingâ€™s sarcoma. A pathologic fracture is present in 10-15% of cases. The soft tissue component of the tumor is rarely calcified. A CT scan is used to delineate the extent of cortical destruction and soft tissue disease. MRI may be used to define the tumor size and its relationship to fascial planes, vessels, and nerves. Finally, metastatic work-up includes a radionucleotide bone scan to evaluate for the presence of metastatic lesions.
The treatment of Ewingâ€™s sarcoma consists of aggressive chemotherapy followed by local surgical resection and radiation therapy if needed. The five-year survival rate is 60-65%. Factors affecting prognosis include the presence of metastasis, tumor size and site, response to therapy, histology, and molecular findings.
In 1979 Askin described a malignant small-cell tumor of the thoracopulmonary region in children (Askin tumor), originating in the soft tissues of the chest wall or peripheral lung which tended to recur locally without signs of dissemination. This neoplasm was included in the category of primitive neuroectodermal tumors (PNET). The diagnosis of this lesion is difficult to determine because of its clinical and morphological similarity to other undifferentiated small, round blue cell tumors as neuroblastoma, extraosseous Ewing's sarcoma, and rhabdomyosarcoma.
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