Abstract
Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other “hits.” These possibilities and the evidence base to support them are explored.
Highlights
Ewing sarcoma (ES) is the second most common malignant bone tumor in adolescents and young adults, leading to more than 200 cases of cancer in the United States per year (SEER)
EWS–FLI1 expression is associated with activation of some genes and repression of others, illustrating the complexity of cellular response to this oncogenic transcription factor (May et al, 1993)
METASTATIC EWING SARCOMA: THE CLINICAL PERSPECTIVE Currently, metastatic disease is clinically defined by the presence of a ES specific translocation in the tissue biopsy of at least one tumor site plus the presence of characteristic lesions in bones, lungs, or malignant cells identified in a staging bone marrow aspirate or biopsy
Summary
Ewing sarcoma (ES) is the second most common malignant bone tumor in adolescents and young adults, leading to more than 200 cases of cancer in the United States per year (SEER). METASTATIC EWING SARCOMA: THE CLINICAL PERSPECTIVE Currently, metastatic disease is clinically defined by the presence of a ES specific translocation in the tissue biopsy of at least one tumor site (primary site) plus the presence of characteristic lesions (by imaging) in bones, lungs, or malignant cells identified in a staging bone marrow aspirate or biopsy.
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