Current Oncology Reports

, Volume 15, Issue 4, pp 378–385

Novel Pathways and Molecular Targets for the Treatment of Sarcoma

  • Ashley E. Frith
  • Angela C. Hirbe
  • Brian A. Van Tine
Sarcomas (SR Patel, Section Editor)

DOI: 10.1007/s11912-013-0319-3

Cite this article as:
Frith, A.E., Hirbe, A.C. & Van Tine, B.A. Curr Oncol Rep (2013) 15: 378. doi:10.1007/s11912-013-0319-3

Abstract

Sarcomas collectively represent over 100 different subtypes of bone and soft tissue tumors of mesenchymal origin. The low response rate to cytotoxic chemotherapies has necessitated the need for development of either histologically driven or pathway-specific targeted therapies. As our understanding of the molecular mechanisms driving certain subtypes is rapidly advancing, the number of targeted therapies is also increasing. Recently identified novel druggable targets include the MDM2 amplifications in well-differentiated and dedifferentiated liposarcomas, the new translocation NAB2:STAT6 of solitary fibrous tumors, the angiopoeitin-TIE2 pathway in angiosarcoma, the suppression of Mcl1 in X:18/synovial sarcomas, the mTOR pathway in malignant peripheral nerve sheath tumors, CDK4 in alveolar rhabdomyosarcoma, cMET regulation in alveolar soft parts sarcoma, the metabolic abnormalities in wild-type/SHD GIST, and the lack of argininosuccinate synthetase 1 expression seen in most sarcomas. It is through a fundamental understanding of sarcoma biology that clinical trials based on molecular targets can be developed.

Keywords

Soft tissue sarcoma Bone sarcoma MDM2 NAB2:STAT6 Angiopoeitin TIE2 Mcl1 mTOR CKD4 cMET SDH ASS1 Succinate dehydrogenase Argininosuccinate Synthetase 1 Liposarcoma Solitary fibrous tumors Angiosarcoma X:18 sarcoma Synovial sarcoma Malignant peripheral nerve sheath tumors Wild-type GIST Alveolar rhabdomyosarcoma Sarcoma 

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Ashley E. Frith
    • 1
  • Angela C. Hirbe
    • 1
  • Brian A. Van Tine
    • 1
    • 2
    • 3
  1. 1.Division of Medical Oncology, Department of Internal MedicineWashington University in St. Louis School of MedicineSt. LouisUSA
  2. 2.Siteman Cancer CenterSt. LouisUSA
  3. 3.Division of Medical Oncology, Department of MedicineWashington University in St. Louis School of MedicineSt. LouisUSA

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