Kazım CEVİZ, Muhammed Emin POLAT, Mecit ÇELİK, Erdem SOBACI, Cavit CEYLAN

Parsiyel Nefrektomi Sonrası Saptanan Anjiyomiyolipomun Nadir Bir Varyantı, Epiteloid Anjiyolipom

Anjiyomiyolipomlar (AML) üç ana yapıdan (vasküler, düz kas ve olgun yağ doku) oluşan benign renal tümörler olarak kabul edilir. AML'nin bileşenlerinin, böbrek mezenkiminin baskılanmamış ve anormal farklılaşmasından kaynaklandığı düşünülmektedir. AML, tüm renal tümörlerin % 2.0- 6.4'ünü oluşturur. Çoğu AML asemptomatiktir görülme sıklığı yaklaşık 10.000 yetişkinde 13'tür. AML’lerin %80’i sporadik, %20’si tüberoz skleroz kompleksinin (TSK) bir parçası olarak gözlenir. Bir AML çeşidi olan Epiteloid AML (EAML), renal hücreli karsinom (RHK) ile karışabilen bir tümör olup benign veya malign (metastaz,nüks) seyredebilen bir tümör çeşididir. EAML görüntülemede de RHK ile karışabilen bir kitledir.Bu nedenle, immünohistokimya olmaksızın RHK’dan ayırıcı tanı yapmak zordur. Biz size 34 yaşında, Bilgisayarlı Tomografide (BT) 11,5 cm renal kitle görülen ve hastaya yapılan parsiyel nefrektomi sonrası patolojide EAML saptanan vakamızı sunuyoruz.

Epithelioid Angiomyolipoma, A Rare Variant of Angiomyolipoma detected After Partial Nephrectomy

Angiomyolipomas (AML) are described as benign renal tumors which consist of vascular, smooth muscle, and adipose tissue components. Abnormal differentiation of renal mesenchyme is the main reason. It forms only 2-6,4% of the whole renal tumors, but it is the most common among the benign tumors. Most of them are asymptomatic and we can see it in almost 13 of 10.000 adults. Eighty percent of the AML is sporadic and 20% of them can be seen as a part of tuberous sclerosis complex (TSC). Epithelioid AML (EAML) is a rare subtype that can have malign or benign progression, it can be misdiagnosed as Renal Cell Carcinoma (RCC) and it is hard to differentiate them. EAML have some common diagnostic characteristics in presentations with other renal tumors. Because of that, we need to make an immunohistochemical examination to differentiate it from RCC, especially. We presented a 34 years old female patient with the pathologic result of the EAMLin the partial nephrectomy material.

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