Large families with apparent Mendelian dominant inheritance are common, but after more than 25 years of extensive searching, only four genes with rare causative mutations have been discovered: fused in sarcoma gene (FUS) (36), GGC repeat expansion in the Notch 2 N-terminal like C gene (NOTCH2NLC) (37), HtrA Serine Peptidase 2 gene (HTRA2) (38), and teneurin transmembrane protein 4 gene (TENM4) (39). Here, TENM4 is linked to sarcoma.