Novel Pathways and Molecular Targets for the Treatment of Sarcoma

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• 作者：Ashley E. Frith (1)
  Angela C. Hirbe (1)
  Brian A. Van Tine (1) (2) (3)
  
• 关键词：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
• 刊名：Current Oncology Reports
• 出版年：2013
• 出版时间：August 2013
• 年：2013
• 卷：15
• 期：4
• 页码：378-385
• 全文大小：183KB
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• 作者单位：Ashley E. Frith (1)
  Angela C. Hirbe (1)
  Brian A. Van Tine (1) (2) (3)
  
  1. Division of Medical Oncology, Department of Internal Medicine, Washington University in St. Louis School of Medicine, St. Louis, MO, 63110, USA
  2. Siteman Cancer Center, St. Louis, MO, 63110, USA
  3. Division of Medical Oncology, Department of Medicine, Washington University in St. Louis School of Medicine, 660 S Euclid, Campus Box 8007, St. Louis, MO, 63110, USA
  

文摘

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.