Berrak Hücreli Renal Tümörler
Özet
Tüm malignitelerin yaklaşık %2 kadarını böbrek kanserleri oluşturur.
Böbrek kanserleri içerisinde berrak hücreli renal hücreli karsinom (RCC), renal karsinomların %65-70 kadarını meydana getirir.
Çoğunlukla 40 yaşından sonra olmak üzere en sık 6. ve 7. dekatta görülür.
Erkeklerde kadınlara göre 1,5 kat daha sıktır.
Olguların %60-80’i insidental olarak radyolojik görüntülemede tanı alır.
Genellikle tek taraflı ve tek odaklı renal kortikal kitle ile karşımıza çıkarlar.
Nefrektomi sonrası 5 yıllık sağ kalım %50-70 iken, metastatik hastalıkta %10’dur.
Bu tümörlerin proksimal kıvrıntılı tübülü döşeyen epitelyal hücreden köken aldığı düşünülmektedir.
Karakteristik olarak von Hippel-Lindau (VHL) gen inaktivasyonuna yol açan kromozom 3p alterasyonları içerirler.
VHL protein kaybı, hipoksi-inducible transkripsiyon faktörü alfa (HIF1α)’nın birikimine yol açar.
HIF1α birikimi hipoksi ilişkili genlerin transkripsiyonuna yol açar.
Mikroskopik olarak tipik berrak sitoplazmaya sahip, dallanan vasküler yapılarla ayrılmış kompakt yuvalar veya asiner büyüme paterni sergileyen tümör izlenir.
Yüksek dereceli tümörlerde rabdoid, sarkomatoid farklılaşma ve tümör nekrozu görülebilir.
Bu halleriyle yüksek dereceli tümörler diğer RCC tiplerine benzer özellikler sergileyip, ayırıcı tanı problemleri yaratabilirler.
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