Park Min S, Araujo Dejka M
Division of Cancer Medicine, University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA.
Curr Opin Oncol. 2009 Jul;21(4):327-31. doi: 10.1097/CCO.0b013e32832c9532.
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.
本综述概述了血管外皮细胞瘤/孤立性纤维性肿瘤（HPC/SFT）谱系肿瘤，重点关注HPC/SFT的组织病理学特征、临床特征、诊断和治疗。
由于HPC/SFT对放疗和细胞毒性化疗相对不敏感，晚期疾病的治疗需要新的疗法。抑制血管生成途径可能为靶向这种恶性肿瘤提供一种新的治疗机制。替莫唑胺和贝伐单抗联合治疗最近已成为HPC/SFT一种潜在的有前景的治疗方案。
鉴于目前正在为软组织肉瘤开发许多新型靶向疗法，需要更好地了解HPC/SFT的分子发病机制和畸变，以确定最佳治疗药物。确定合适的靶点并设计合理的前瞻性临床试验不仅将改善HPC/SFT的治疗，还将引领个性化、靶向治疗的新范式。
