Despite some data suggesting that heterozygous germline variants in RECQL4 increase risk of osteosarcoma, in this analysis of an international registry of RTS II patients and their family members, investigators found that RTS II family members with heterozygous germline pathogenic variant in RECQL4 did not have an increased risk of developing cancer, compared to the age-adjusted population estimate per the Surveillance, Epidemiology, and End Results (SEER) program (Maciaszek et al., 2019; Martin-Giacalone et al., 2022). Here, RECQL4 is linked to osteosarcoma.