Min S Park1, Dejka M Araujo. 1. Division of Cancer Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA.
Abstract
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.
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.
Authors: Mihai Hajdu; Samuel Singer; Robert G Maki; Gary K Schwartz; Mary Lou Keohan; Cristina R Antonescu Journal: J Pathol Date: 2010-07 Impact factor: 7.996
Authors: Min S Park; Shreyaskumar R Patel; Joseph A Ludwig; Jonathan C Trent; Charles A Conrad; Alexander J Lazar; Wei-Lien Wang; Piyaporn Boonsirikamchai; Haesun Choi; Xuemei Wang; Robert S Benjamin; Dejka M Araujo Journal: Cancer Date: 2011-04-08 Impact factor: 6.860
Authors: Sarah Bouhabel; Guy Leblanc; Jose Ferreira; Yves E Leclerc; Pierre Dubé; Lucas Sidéris Journal: World J Surg Oncol Date: 2011-10-31 Impact factor: 2.754
Authors: Breelyn A Wilky; Elizabeth A Montgomery; Angela A Guzzetta; Nita Ahuja; Christian F Meyer Journal: Ann Surg Oncol Date: 2013-09-18 Impact factor: 5.344