Böbreğin Diğer Sarkomları
Özet
Primer renal sarkomlar, böbreğin nadir görülen malign mezenkimal tümörleridir ve leiyomyosarkom, sinovyal sarkom, soliter fibröz tümör, Ewing sarkomu, sklerozan epiteloid fibrosarkom ve anaplastik sarkom (DICER1 ilişkili) gibi birçok alt tipi içerir. Leiyomyosarkom, en sık görülen alt tiptir; genellikle renal ven veya hilustan köken alır ve orta yaşlı kadınlarda daha sık izlenir. Eozinofilik sitoplazmalı, puro şeklinde nükleuslu iğsi hücrelerden oluşur; SMA, desmin ve h-kaldesmon pozitiftir. Prognoz kötü olup metastaz sıktır. Sinovyal sarkom, genç erişkinlerde görülür, SS18::SSX1/2 füzyonu gösterir ve TLE1, BCL2 ile pozitiftir; çoğu monofazik iğsi hücreli formdadır. Soliter fibröz tümör (SFT), hemanjioperisitik damarlar içeren, değişken selülariteye sahip lezyonlardır; STAT6 ve CD34 pozitiftir ve genellikle benign seyreder. Renal Ewing sarkomu, genç erişkinlerde görülür; EWSR1::FLI1 veya ETV4 füzyonları taşır ve NKX2.2 ile CD99 pozitiftir; agresif seyirlidir. Sklerozan epiteloid fibrosarkom, EWSR1::CREB3L1 füzyonu ve MUC4 pozitifliği ile karakterizedir. Anaplastik renal sarkom (DICER1 ilişkili) ise çocuklarda, sıklıkla kistik nefrom zemininde gelişir ve DICER1 mutasyonu taşır. Tedavide cerrahi esastır; bazı tiplerde kemoterapi eklenir. Prognoz, histolojik alt tipe ve moleküler değişikliklere bağlıdır.
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