A case report of renal Ewing sarcoma

Year 2012, , 127 - 129, 01.03.2012

Alaaddin Akay Gökmen Akarer Zafer Akar Gül Türkçü Taner Çiftci

https://doi.org/10.5799/ahinjs.01.2012.01.0129

Abstract

Primitive neuro-ectodermal tumors (PNETs) are seen mostly at central nervous system. Peripheral localizations are rare. PNETs are belong to Ewing sarcoma family. Ewing sarcoma has two subtypes as originated from bones or from another organ. Renal PNET is a very rare tumor. Peripherally localized PNETs originated from kidney, usually found at young men, and have poor prognosis with aggressive behavior. A 28-year-old man referred to our clinic with left flank pain and gross hematuria. Radiological investigations yielded gross renal mass compatible with malignancy. Radical nephrectomy was performed. Histopathological diagnosis was PNET/Ewing sarcoma with findings of large and hyperchromatous nuclei, narrow cytoplasms, spindle shaped cells- but mostly rosette formation- and strong straining with CD99. Chemotherapy was given to the patient. At the end of the chemotherapy, there no recurrence was detected by radiological investigations.

Keywords

Ewing sarcoma, renal tumor, case

Böbrek Ewing sarkomu olgu sunumu

Abstract

Primitif nöroektodermal tümörler (PNET) en çok santral sinir sisteminde görülürler. Periferik yerleşimleri ise oldukça nadirdir. PNET Ewing sarkom ailesindendir. Ewing sarkom kemik ve kemik dışı kökenli olmak üzere iki grupta değerlendirilir. Renal PNET oldukça nadir görülür. Renal kökenli periferik yerleşimli PNET\'ler genelde genç erişkinlerde görülür, agresif seyirli bir klinik tablo ile ilerler ve kötü prognoza sahiptir. Yirmi sekiz yaşında erkek hasta kliniğimize gross hematüri ve sol yan ağrısıyla başvurdu. Radyolojik değerlendirmesinde malign böbrek tümörü ile uyumlu 10x12 cm boyutlarında renal kitle saptandı. Hastaya radikal nefrektomi yapıldı. Patolojik incelemede iri, hiperkromatik çekirdekli, dar stoplazmalı, bir kısmı iğsi görünümde çoğu alanlarda rozet oluşturan, CD99 ile yaygın ve güçlü boyanma gösteren, PNET/Ewing sarkom tanısı konuldu. Hastaya kemoterapi planlandı. Kemoterapi bitiminde hastanın radyolojik kontrollerinde herhangi bir nükse rastlanmadı.

Keywords

Ewing sarkom, renal tümör, olgu

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