Türk Nöroşirürji Dergisi 2017 , Vol 27 , Num 2
Neurocutaneous Syndromes-Phacomatoses
Erkut Baha BULDUK1,Alp Özgün BÖRCEK1
1Gazi Üniversitesi, Tıp Fakültesi, Pediatrik Nöroşirürji Bilim Dalı, Ankara, Türkiye Neurocutaneus syndromes are genetically inherited diseases that involve multiple tissues of ectodermal origin. Due to their skin manifestations, these syndromes are also called phacomatoses. Up to 67 of these syndromes, which are associated with tumors ranging from hamartomas to malignant carcinomas, have been described. Therefore, they are a very important issue in neurooncology. The aim of this paper is to summarize the clinical outlines of the most common neurocutaneus syndromes in the light of recent developments.

Clinical outlines of the six most common neurocutaneous syndromes (Neurofibromatosis type I, Neurofibromatosis type II, Tuberous sclerosis, Sturge-Weber syndrome, Von Hippel Lindau syndrome and Ataxia-telengiectasia) will be discussed in detail. Neurocutaneous syndromes (phacomatoses) result in cancer predisposition and, therefore, are important diseases in daily neurooncological practice. Anahtar Kelimeler : Neurofibromatosis, Tuberous Sclerosis, Sturge-Weber syndrome, Von Hippel Lindau syndrome, Ataxia-Telengiectasia