Kranial Hemanjıoblastoma; Von Hıppel-Lındau Sendromu Olgusu
Özet
Von Hippel-Lindau (VHL) sendromu, otozomal dominant geçişli, VHL gen mutasyonu sonucu gelişen ve çoklu tümörlerle karakterize edilen nadir bir genetik hastalıktır. VHL sendromunda santral sinir sisteminde en sık görülen tümörler beyin hemanjiyoblastomalarıdır. Bu tümörler özellikle serebellum, beyin sapı ve omurilikte yerleşim gösterir ve yüksek vaskülariteye sahip benign lezyonlardır. VHL sendromlu olgunun serebellumunda iki adet farklı lokalizasyondaki hemanjiyoblastomayı literatür eşliğinde sunuyoruz.
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