title: Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor.
abstract: OBJECTIVE
  This review highlights the current body of knowledge regarding the role of the vascular endothelial growth factor (VEGF) and its receptor (VEGFR) in angiosarcoma, epithelioid hemangioendothelioma (EHE), and hemangiopericytoma/solitary fibrous tumor (HPC/SFT). Therapeutic agents that target this pathway are reviewed.
  
  
  RESULTS
  Several phase II trials in advanced soft tissue sarcoma patients have investigated the efficacy of bevacizumab, an anti-VEGF antibody, as well as sunitinib, sorafenib, and pazopanib, VEGFR tyrosine kinase inhibitors (TKIs). Although response rates and progression-free survival periods were generally low, several angiosarcoma, EHE, and HPC/SFT patients demonstrated response or durable disease stabilization on these therapies. Biological mechanisms underlying the activity of these agents in angiosarcoma, EHE, and HPC/SFT are poorly understood. Some angiosarcoma tumors, however, harbor specific activating mutations in VEGFR2, which may be effectively targeted by VEGFR TKIs.
  
  
  CONCLUSIONS
  Inhibition of the VEGF/VEGFR pathway may be a rational and effective therapy for certain patients with angiosarcoma, EHE, and HPC/SFT, but more studies are needed to confirm these findings and to identify which patients will benefit from these agents.
authors.affiliation: Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA.
authors.forename: Min S
authors.initials: MS
authors.lastname: Park
authors.forename: Vinod
authors.initials: V
authors.lastname: Ravi
authors.forename: Dejka M
authors.initials: DM
authors.lastname: Araujo
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D018323:Hemangioendothelioma, Epithelioid / Q000188:drug therapy / Q000503:physiopathology*; D006394:Hemangiosarcoma / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D040262:Receptors, Vascular Endothelial Growth Factor / Q000502:physiology*; D015398:Signal Transduction / Q000187:drug effects / Q000502:physiology*; D054364:Solitary Fibrous Tumors / Q000188:drug therapy / Q000503:physiopathology*; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000502:physiology*
chemical_list: D000970:Antineoplastic Agents; D042461:Vascular Endothelial Growth Factor A; D040262:Receptors, Vascular Endothelial Growth Factor