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¹Şişli Memorial Hastanesi, Nöroşirürji Bölümü, İstanbul, Türkiye
²Nöroakademi Grup, İstanbul, Türkiye In 1942 Stout and Murray described hemangiopericytoma and solitary fibrous tumor of the pleura. Subsequently these two tumors were described in various parts of the body. This review aims to summarize current understanding of the tumor biology of these two rare brain tumors and deliniate targeted treatment strategies. First, recent studies on the molecular biology of these two tumors will be mentioned. This will be followed by targeted drug strategies. Recent studies have revealed that these two tumors harbor similar chromosomal translocations. These translocations result in abnormal STAT 6 transcription factor activity, which in turn leads to antiapoptotic bcl-2 overexpression as well as PDGF and telomerase overactivity. Today, there are targeted drugs for all of these signaling intermediates (teriflunomide, venetoclax, sunitinib, imetelstat). Molecular biological studies on these two rare tumors create novel targets for pharmacotherapy. Anahtar Kelimeler : Solitary fibrous tumor, Hemangiopericytoma, Targeted treatment