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.