SIX3 and extraskeletal myxoid chondrosarcoma: SIX3 regulates the transcriptional activity of the orphan nuclear receptor NOR-1 (NR4A3), which regulates the survival of cells, and acts as a part of the EWS/NOR-1 fusion protein, implicating the oncogenesis of human extraskeletal myxoid chondrosarcoma (EMC) [59].