Systemic treatment of soft-tissue sarcoma-gold standard and novel therapies

Nat Rev Clin Oncol. 2014 Apr;11(4):187-202. doi: 10.1038/nrclinonc.2014.26. Epub 2014 Mar 18.


Soft-tissue sarcoma (STS) is a rare and heterogeneous group of tumours that comprise approximately 1% of all adult cancers, and encompass over 50 different subtypes. These tumours exhibit a wide range of differing behaviours and underlying molecular pathologies, and can arise anywhere in the body. Surgical resection is critical to the management of locoregional disease. In the locally advanced or metastatic disease settings, systemic therapy has an important role in the multidisciplinary management of sarcoma. Cytotoxic therapy that usually consists of doxorubicin and ifosfamide has been the mainstay of treatment for many years. However recent advances in molecular pathogenesis, the development of novel targeted therapies, changes in clinical trial design and increased international collaboration have led to the development of histology-driven therapy. Furthermore, genomic profiling has highlighted that some STS are driven by translocation, mutation or amplification and others have more complex and chaotic karyotypes. In this Review, we aim to describe the current gold standard treatment for specific STS subtypes as well as outline future promising therapies in the pipeline.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Algorithms
  • Antibodies, Monoclonal / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cancer Vaccines / therapeutic use
  • Combined Modality Therapy
  • Drug Resistance, Neoplasm / drug effects
  • Histone Deacetylase Inhibitors / therapeutic use
  • Humans
  • Immunotherapy
  • Immunotherapy, Adoptive
  • Molecular Targeted Therapy
  • Prodrugs / administration & dosage
  • Protein Kinase Inhibitors / pharmacology
  • Protein Kinase Inhibitors / therapeutic use
  • Proto-Oncogene Proteins c-mdm2 / antagonists & inhibitors
  • Sarcoma / drug therapy*
  • Sarcoma / genetics
  • Sarcoma / pathology
  • Sarcoma / therapy


  • Antibodies, Monoclonal
  • Cancer Vaccines
  • Histone Deacetylase Inhibitors
  • Prodrugs
  • Protein Kinase Inhibitors
  • MDM2 protein, human
  • Proto-Oncogene Proteins c-mdm2