These data led us to evaluate a panel of SNPs in germline ERCC genes in a cohort of STS, considering that the alternative allele for SNP rs11615 in the ERCC1 gene and the alternative allele for SNPs (rs13181and rs1799793) in the ERCC2 gene has a higher frequency in STS patients versus the distribution in the general healthy population and NER pathway is deeply affected since 97% (30 out of 31) of STS patients have at least one variation in ERCC genes. Here, ERCC2 is linked to telomere syndrome.