Although germline CHEK2 P/LP variants were more common in individuals with DFSP, a large number of individuals with CHEK2 P/LP variants in Geisinger and UK Biobank did not develop DFSP suggesting that CHEK2, if contributing to the formation of this cancer, is a low-risk susceptibility gene that acts in combination with other factors to increase risk. The gene discussed is CHEK2; the disease is dermatofibrosarcoma protuberans.