Genetically distinct from osseous CS is the extraskeletal myxoid CS, which is characterized by the chromosomal translocation t(9;22)(q22;q12), or more rarely t(9;17)(q22;q11) and t(9;15)(q22;q21), involving the nuclear receptor subfamily 4, group A (NR4A3) and giving rise to the fusion genes EWS::NR4A3, TAF15::NR4A3, and TCF12::NR4A3, respectively [11]. Here, TCF12 is linked to Cowden syndrome 1.