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¹Pursaklar Devlet Hastanesi, Beyin ve Sinir Cerrahisi Kliniği, Ankara, Türkiye
²SBÜ, Dışkapı Yıldırım Beyazıt Eğitim ve Araştırma Hastanesi, Beyin ve Sinir Cerrahisi Kliniği, Ankara, Türkiye Pleomorphic xanthoastrocytoma (PXA) is a rare and slow growing tumor that accounts for less than 1% of all central nervous system astrocytomas. They were classified as "pleomorphic xanthoastrocytoma Grade 2" tumors under the title of "other astrocytic tumors" in "the 2016 World Health Organization Classification of Tumors of the Central Nervous System". Mitosis increase and necrosis are seen in some of the PCAs. This group of PXAs has been classified as "anaplastic pleomorphic xanthoastrocytoma (aPXA) Grade 3" in the new classification. Pleomorphic xanthoastrocytoma is a low-grade glioma that is thought to be caused by subpial astrocytes due to superficial locations and excessive reticular fibers. In a young patient, PXA should be considered primarily in the presence of a tumor which is consistent with the clinical findings, has a cystic component, shows contrast enhancement on imaging, has an immunohistochemical profile of BRAFV600E positive, and is IDH1 negative and morphologically a pleomorphic astrocytoma. In this review, information about the clinical features, magnetic resonance imaging scans, molecular studies, tumor biology, diagnosis and treatment models of PXAs are presented according to recent studies. Anahtar Kelimeler : Pleomorphic xanthoastrocytoma, Central nervous system, Pediatric, Adult