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Table 9. Agents With Selective Activity Against Subtypes of Soft Tissue Tumors

Agent Soft Tissue Sarcoma Subtype 
Sunitinib [12,13]Alveolar soft part sarcoma
Cediranib [14]Alveolar soft part sarcoma
Sunitinib [15]Solitary fibrous tumor
Sirolimus [16]Perivascular epithelioid cell tumor (PEComa)
Crizotinib [17]Inflammatory myofibroblastic tumor
ImatinibTenosynovial giant cell tumor
Imatinib [18]Chordoma
Imatinib [19]Dermatofibrosarcoma protuberans


  1. Stacchiotti S, Negri T, Zaffaroni N, et al.: Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 22 (7): 1682-90, 2011.  [PUBMED Abstract]

  2. Stacchiotti S, Tamborini E, Marrari A, et al.: Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res 15 (3): 1096-104, 2009.  [PUBMED Abstract]

  3. Kummar S, Allen D, Monks A, et al.: Cediranib for metastatic alveolar soft part sarcoma. J Clin Oncol 31 (18): 2296-302, 2013.  [PUBMED Abstract]

  4. Stacchiotti S, Negri T, Libertini M, et al.: Sunitinib malate in solitary fibrous tumor (SFT). Ann Oncol 23 (12): 3171-9, 2012.  [PUBMED Abstract]

  5. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, et al.: Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 28 (5): 835-40, 2010.  [PUBMED Abstract]

  6. Butrynski JE, D'Adamo DR, Hornick JL, et al.: Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 363 (18): 1727-33, 2010.  [PUBMED Abstract]

  7. Stacchiotti S, Longhi A, Ferraresi V, et al.: Phase II study of imatinib in advanced chordoma. J Clin Oncol 30 (9): 914-20, 2012.  [PUBMED Abstract]

  8. Rutkowski P, Van Glabbeke M, Rankin CJ, et al.: Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 28 (10): 1772-9, 2010.  [PUBMED Abstract]