New insights into the hemangiopericytoma/solitary fibrous tumor spectrum of tumors
- 1 July 2009
- journal article
- review article
- Published by Ovid Technologies (Wolters Kluwer Health) in Current Opinion in Oncology
- Vol. 21 (4), 327-331
- https://doi.org/10.1097/cco.0b013e32832c9532
Abstract
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. 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. 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.Keywords
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