Analysis in the Prospective Lynch Syndrome Database identifies sarcoma as part of the Lynch syndrome tumor spectrum

We conclude that the incidence of OS in LS patients is significantly increased compared to the general population.

We suggest that osteosarcoma is part of the LS spectrum in adults and seems to be associated particularly with the path_MSH2 genotype. The association of STS with path_MMR variants is currently less clear, although there are case reports on young LS carriers with soft tissue sarcomas.

https://onlinelibrary.wiley.com/doi/10.1002/ijc.33214