Yetişkin Böbreğinde Mezenşim Kökenli Tümörler-Klasik Anjiomyolipoma/ Pecoma
Özet
Klasik anjiyomiyolipom (AML), diğer adıyla perivasküler epiteloid hücreli tümör (PEComa), değişen oranlarda yağ dokusu, düz kas hücreleri ve kalın duvarlı damarlar içeren benign bir mezenkimal tümördür. Böbrek tümörlerinin yaklaşık %1’ini oluşturur. Sporadik olgular genellikle yaşlı kadınlarda tek taraflı ve soliterdir, tüberoskleroz zemininde gelişen olgular ise sıklıkla bilateral ve multifokaldir. Patogenezde, TSC1 veya TSC2 genlerinde biallelik inaktivasyon sonucu mTOR yolağının aşırı aktivasyonu gelişir ve bu da hücre proliferasyonunu artırır. Histolojik olarak AML, adipöz doku, iğsi veya epiteloid düz kas hücreleri ve displastik kalın duvarlı damarlar içerir. İmmünohistokimyasal olarak HMB45, Melan-A, Katepsin K, SMA ve Desmin pozitif, keratin negatiftir. Bu boyanma paterni myomelanositik farklılaşmayı doğrular. Klinik olarak çoğu olgu asemptomatiktir, ancak yan ağrısı, hematüri veya retroperitoneal hemoraji (Wunderlich sendromu) ile ortaya çıkabilir. Tedavide cerrahi eksizyon ve anjiyografik embolizasyon uygulanır; büyük veya çoklu lezyonlarda mTOR inhibitörleri etkili olabilir. AML genellikle benign seyirli olmasına rağmen, nadiren sarkomatöz transformasyon ve metastaz bildirilmiştir; bu nedenle uzun dönem takip önerilir.
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