Herediter Adenomatöz Polipozis Sendromlarında Profilaktik Cerrahi: Ne Zaman ve Nasıl? – Güncel Yaklaşımlar ve Literatür Derlemesi

Yıl 2020, , 528 - 535, 15.09.2020

Emrah Akın , Furkan Kucuk Fatih Altıntoprak

https://doi.org/10.31832/smj.734079

Öz

Herediter adenomatöz polipozis sendromları; Familyal Adenomatöz Polipozis (FAP), Atenüe Familyal Adenomatöz Polipozis (AFAP), MUTHY ilişkili Adenomatöz Polipozis (MAP) ve Polimeraz Proofreading Sendrom (PPS) olarak tanımlanan farklı hastalıkları bünyesinde barındırmaktadır. Hastalar fenotip özellikleri, genotip ilişkili presentasyonları, yaş, cerrahinin zamanlaması, kapsamı ve tekniği gibi çeşitli faktörlerin bir arada değerlendirildiği profilaktik cerrahi için adaydır. Günümüzde klinik, moleküler ve teknik ilerlemeler daha az invaziz ve riski uyarlanmış cerrahi ile birlikte bireyselleştirilmiş tedavi seçeneklerini sunmaktadır. Malignite gelişmeden önce hedef organın ortadan kaldırılması olarak tanımlanan proflaktik cerrahi seçeneği bu sendromlarda gündeme gelmektedir. Bu yazıda; tanımlanan sendromlardaki genetik altyapı ve klinik prezentasyon farkları gözden geçirilip, proflaktik cerrahinin zamanlaması ve seçenekleri güncel literatür verileri ışığında derlenmiştir.

Anahtar Kelimeler

FAP, profilaktik cerrahi, herediter polipozis sendromlar

Kaynakça

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