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Chondrosarcoma, Extraskeletal Myxoid [Supplementary Concept]

A rare malignant soft tissue neoplasm that originates from CHONDROBLASTS. It is most common in male middle-aged and elderly individuals and often presents as deep lesions in soft tissue of the extremities. Malignancy is low, but recurrence and METASTASIS can occur many years after the initial diagnosis. Histologically, the tumors consist of a mixture of cellular and myxoid stromal components. Can be caused by somatic chromosomal translocations resulting in fusions between the NR4A3 and EWSR1 genes, and NR4A3 and TAF15 genes. OMIM: 612237

Date introduced: November 5, 2012

MeSH Unique ID: C563195

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