New insights into the hemangiopericytoma/solitary fibrous tumor spectrum of tumors.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Park
Min S
MS
Division of Cancer Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA.
Araujo
Dejka M
DM
D006393:Hemangiopericytoma / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D054364:Solitary Fibrous Tumors / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy*