LAUSR

Extraskeletal myxoid chondrosarcoma (EMC) is an ultra-rare sarcoma of uncertain differentiation. Despite its name, EMC does not show a cartilaginous differentiation and has nothing to do with other chondrosarcoma subtypes. Nevertheless, similarly to conventional chondrosarcoma, EMC for a long time has been considered resistant to systemic treatments and radiotherapy. The identification of a chromosomal translocation involving the NR4A3 gene, which can be rearranged with different partners, at the beginning of the last decade, has made it possible to distinguish this specific sarcoma from other mimics (e.g., malignant myoepitelioma). EMC is considered to be an indolent tumor, but although surgery remains the only curative therapy in localized cases, available longterm follow-up data are scanty and refer to series not molecularly confirmed. In addition, the role of systemic treatment of EMC plays in the metastatic setting is poorly defined. PRESENT