Knowra Ewing sarcoma Ewing sarcoma Ewing sarcoma is a malignant small round-cell tumor that usually arises in bone or soft tissue. Most cases are driven by an EWSR1–ETS gene fusion, especially EWSR1::FLI1.
EWSR1::FLI1 : A fusion protein produced by joining EWSR1 and FLI1 genes, found in most Ewing sarcomas. This defining fusion rewires gene expression and drives most tumors.
Small round-cell tumor : A group of tumors composed of relatively uniform, small, round cells with scant cytoplasm. Ewing sarcoma belongs to this diagnostic group, whose members can look alike under a microscope.
Fluorescence in situ hybridization : A method that uses fluorescent DNA probes to detect specific sequences or chromosomal rearrangements in cells. It can reveal rearrangements involving EWSR1 in tumor samples.
CIC-rearranged sarcoma : An undifferentiated sarcoma commonly driven by CIC gene fusions, often involving DUX4. It can resemble Ewing sarcoma morphologically but has a distinct molecular driver.
Ewing sarcoma metastasis : The spread of Ewing sarcoma from its primary site to distant locations, especially lungs, bones, or bone marrow. Metastatic disease at diagnosis is among the strongest predictors of outcome.
EWSR1 gene : A gene encoding a protein involved in RNA processing and transcriptional regulation. Its rearrangement supplies the EWSR1 portion of the characteristic fusion.
Bone tumor : An abnormal growth arising in bone, which may be benign or malignant. Many Ewing sarcomas begin in bone, especially in children and young adults.
RNA sequencing : A sequencing method that measures RNA molecules and can identify expressed gene fusions. It can identify the fusion partner when a rearrangement needs precise classification.
BCOR-altered sarcoma : A group of sarcomas defined by BCOR alterations, including BCOR::CCNB3 fusion. This round-cell tumor can mimic Ewing sarcoma yet belongs to a separate molecular entity.
Cancer prognosis : An estimate of a cancer’s likely course and outcome based on disease and patient factors. Survival varies sharply with metastatic status, tumor location, and response to treatment.
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