Germline predisposition to soft tissue sarcoma

Soft tissue sarcoma (STS) most often occurs sporadically, but can also arise in the setting of a germline cancer predisposition syndrome (CPS). There is significant diversity amongst STS diagnoses as these tumors exhibit variety histologies, occur all age groups, and any location body. This reflected many known associated associations. Some diagnoses, such anaplastic rhabdomyosarcoma, are with high heritability other STS, Ewing sarcoma, notably absent from CPS. Recognizing when more likely to be hereditary influence clinical management. Individuals diagnosed due CPS may at risk for malignancies should undergo additional surveillance early detection. Additionally, family members genetic testing they develop CPS-associated malignancies. underlying have implications treatment concurrent malignancy case PARP inhibitor therapy homologous recombination deficiency. review summarizes current knowledge selected their associations