Despite its limited involvement in tumorigenesis, changes in ASXL3 are implicated in developmental defects, congenital heart disease, and Bainbridge–Ropers syndrome (BRS) [60,61,62], which shares similarities with BOS, caused by autosomal truncations in ASXL1 [63,64]. Here, ASXL3 is linked to Buschke-Ollendorff syndrome.