SMC3 and Cornelia de Lange syndrome: To note, patients affected by the Cornelia de Lange Syndrome (CdLS) caused by germline mutations in cohesin genes (∼65% NIPBL, ∼5% SMC1A and HDAC8, 1–2% SMC3 and RAD21) (Mannini et al., 2013; Singh and Gerton, 2015), rarely develop cancers and likely not for genomic instability but as a consequence of their clinical features (e.g., gastric reflux) (Schrier et al., 2011; Deardorff et al., 2012).