Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor.

Abstract

PURPOSE OF REVIEW:

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.

RECENT FINDINGS:

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.

SUMMARY:

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: Min S Park, Vinod Ravi, Dejka M Araujo
• Journal: Current opinion in oncology (Curr Opin Oncol) Vol. 22 Issue 4 Pg. 351-5 (Jul 2010) ISSN: 1531-703X [Electronic] United States
• PMID: 20485168 (Publication Type: Journal Article, Review)

Chemical References

• Antineoplastic Agents
• Vascular Endothelial Growth Factor A
• Receptors, Vascular Endothelial Growth Factor

Topics

• Antineoplastic Agents (therapeutic use)
• Hemangioendothelioma, Epithelioid (drug therapy, physiopathology)
• Hemangiosarcoma (drug therapy, physiopathology)
• Humans
• Receptors, Vascular Endothelial Growth Factor (physiology)
• Signal Transduction (drug effects, physiology)
• Solitary Fibrous Tumors (drug therapy, physiopathology)
• Treatment Outcome
• Vascular Endothelial Growth Factor A (physiology)