Journal
CURRENT OPINION IN ONCOLOGY
Volume 21, Issue 4, Pages 327-331Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/CCO.0b013e32832c9532
Keywords
antiangiogenesis; hemangiopericytoma; solitary fibrous tumor; targeted therapy; vascular endothelial growth factor
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Purpose of review This review provides an overview of the hemangiopericytoma/solitary fibrous tumor (HPC/SFT) spectrum of tumors, focusing on the histopathologic characteristics, clinical features, diagnosis, and treatment of HPC/SFT. Recent findings Due to the relatively insensitive nature of HPC/SFT to radiotherapy and cytotoxic chemotherapy, new therapies are needed for treatment of advanced disease. Inhibition of angiogenic pathways may provide a novel therapeutic mechanism for targeting this malignancy. Combination therapy with temozolomide and bevacizumab has recently emerged as a potentially promising regimen for HPC/SFT. Summary With many novel targeted therapies currently in development for soft tissue sarcomas, a better understanding of the molecular pathogenesis and aberrations of HPC/SFT is needed to determine optimal therapeutic agents. Identifying appropriate targets and designing rational prospective clinical trials will not only improve treatment of HPC/SFT but will also lead to a new paradigm of personalized, targeted therapy.
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