Sarcomas are a heterogeneous group of uncommon mesenchymal tumours that arise from bone and soft tissue. They account for 1% of cancers in adults and comprise more than 70 different histological subtypes [1]. Sarcomas are extremely varied, with differences in clinical behaviour, driver molecular aberrations, and genetic complexity. The pathogenesis of sarcomas is multifactorial and likely involves a complex interplay of environment, lifestyle, and germline genetics. With the advent of next-generation sequencing (NGS) technology and genome-wide association studies (GWASs), there have been significant improvements in our understanding of the development of sarcomas and possible predisposition loci. Recent genomic investigations indicate that up to 25% of patients with soft tissue and bone sarcomas may have an underlying germline genetic predisposition syndrome [2]. Knowledge of germline genetic changes in the development of sarcoma is evolving, with progressively greater understanding of the genetic background in sarcomagenesis.