Clear Cell Sarcoma of the Kidney: A Remarkably Uncommon Case Report

Öz

Clear cell sarcoma of the kidney (CCSK) is one of the most common malignant renal neoplasms in childhood, although it is quite rare. Its incidence peaks around the age of 3 and it is twice as common in males. We present a rare case of CCSK showing a rare histopathological pattern in terms of age and gender. A 7-month-old female patient presented with complaints of vomiting, abdominal swelling, and diarrhea. Imaging analysis revealed a mass in the right kidney, and right radical nephrectomy was performed, considering Wilms tumor (WT). There was a solid gray-white mass that completely filled the kidney, with a maximum dimension of 8.5 cm, macroscopically. The samples obtained showed intratubular structures within the tumor and kidney parenchyma in limited areas at the periphery. The tumor had a normochromic monotonous nucleus with occasional clear cytoplasm and mostly exhibited a palisade-like arrangement pattern. In the differential diagnosis, with blastemal WT and Ewing sarcoma being the primary considerations, the positivity of CyclinD1 markers, negativity of other markers, and morphological characteristics were evaluated in favor of CCSK with a palisaded Schwannian pattern. The patient was put on an intensive chemotherapy process, but was lost after relaps at weet 24. CCSK is seen in a similar age group as WT but is distinguished by its rarity and relatively worse prognosis. Histopathologically, it most commonly presents in a myxoid pattern and least commonly in anaplastic pattern. The palisaded schwannian type seen in our case is recorded at a rate of 11%. No spesific diagnostic marker has been identified immunohistochemically, but the overexpression of markers such as CyclinD1, BCOR, and EZH2 is reported to be helpful in diagnosis. Due to its high metastatic potential and limited treatment options, further research is needed to understand the molecular nature of the disease.

Anahtar Kelimeler

• Clear cell sarcoma of the kidney
• Case report
• Rare tumor of the childhood
• Malignant renal tumor of the childhood

Böbreğin Berrak Hücreli Sarkomu: Oldukça Nadir Bir Olgu Sunumu

Öz

Böbreğin berrak hücreli sarkomu (BBHS) oldukça nadir görülmekle birlikte çocukluk çağının en sık görülen malign böbrek neoplazilerinden biridir. İnsidansı 3 yaş civarında pik yapmakta olup erkek cinsiyette 2 kat daha sık görülmektedir. Nadir bir yaş ve cinsiyette nadir bir histopatolojik patern gösteren BBHS olgusu sunduk. 7 aylık kız hasta kusma, karında şişlik ve ishal şikayeti ile başvurdu. Yapılan görüntüleme analizinde sağ böbrekte kitle saptanması üzerine Wilms tümörü (WT) düşünülerek sağ radikal nefrektomi uygulandı. Makroskopik olarak en büyük boyutu 8,5 cm olan böbreğin tamamını dolduran solid gri-beyaz kitle mevcuttu. Alınan örneklerde tümör içerisinde entrape tübül yapıları ve periferde sınırlı alanlarda böbrek parankimi seçilmekteydi. Tümör normokromatik monoton nükleuslu yer yer şeffaf sitoplazmalı ve çoğunlukla palizat benzeri dizilim paterninde idi. Ayırıcı tanıda başta blastemal WT ve Ewing sarkomu da düşünülerek yapılan belirteçlerden SiklinD1 pozitifliği, diğer markırların negatifliği ve morfolojik özellikleri ile olgu palizatlanan schwannian paternde BBHS lehine değerlendirildi. Olgu yoğun kemoterapi sürecine alındı ancak 24. hafta relaps sonrası kaybedildi. BBHS, WT ile benzer yaş grubunda görülmekle birlikte oldukça nadir olması ve nispeten daha kötü prognozlu olması ile ayrılmaktadır. Histopatolojik olarak en sık miksoid, en az anaplastik paternde karşımıza çıkmaktadır. Olgumuzda görülen palizatlanan schwannian tip ise %11 oranında kaydedilmiştir. İmmunohistokimyasal olarak spesifik tanı koydurucu bir belirteç henüz tanımlanmamış olup SiklinD1, BCOR, EZH2 gibi markırların overekspresyonunun tanıya yardımcı olduğu bildirilmektedir. Metastaz potansiyeli yüksek ve tedavi alternatifi kısıtlı olması nedeni ile hastalığın moleküler doğasını anlamak için yeni araştırmalara ihtiyaç vardır.

Anahtar Kelimeler

• Böbreğin şeffaf hücreli sarkomu
• olgu sunumu
• Çocukluk çağının nadir tümörü
• çocukluk çağı malign böbrek tümörü

Kaynakça

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