Zafer AKAR, Taner ÇİFTCİ, Alaaddin AKAY, Gül TÜRKÇÜ, Gökmen AKARER

Böbrek Ewing sarkomu olgu sunumu

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.

A case report of renal Ewing sarcoma

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.

PDF

___

1. Casella R, Moch H, Rochlitz C et al. Metastatic primitive neuroectodermal tumor of the kidney in adults. Eur Urol 2001; 39(5):613-7.
2. Craft A, Cotterill S, Malcolm A, et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: the second United Kingdom Children’s Cancer Study Group and the Medical Research Council Ewing’s Tumor Study. J Clin Oncol 1998; 16(11): 3628-33.
3. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of the bone. N Engl J Med 2003; 348(8):694-701.
4. Rodriguez-Galindo C, Marina NM, Fletcher BD, et al: Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer 1997; 79(11): 2243-50.
5. Parham DM, Roloson GJ, Feely M, et al. Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms’ Tumor Study Group Pathology Center. Am J Surg Pathol 2001; 25(2): 133-46.
6. Gupta RK, Soni SM, Joshi VV, et al: Primary PNET of kidney: report of two cases and review of literature. Indian J Cancer 2000; 37(4): 184-9.
7. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003; 348(8): 694-701.
8. Bacci G, Ferrari S, Longhi A, et al. Role of surgery in local treatment of Ewing’s sarcoma of the extremities in patients undergoing adjuvant and neoadjuvant chemotherapy. Oncol Rep 2004; 11(1): 111-20.
9. Kuroda M, Urano M, Abe M, et al. Primary primitive neuroectodermal tumor of the kidney. Pathol Int 2000; 50(12): 967-72.
10. Craft A, Cotterill S, Malcolm A, et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: the second United Kingdom Children’s Cancer Study Group and the Medical Research Council Ewing’s Tumor Study. J Clin Oncol 1998; 16(11): 3628-33.
11. Burgert EO Jr, Nesbit ME, Garnsey LA et al. Multimodal therapy for the management of nonpelvic, localized Ewing’s sarcoma of bone: intergroup study IESS-II. J Clin Oncol 1990; 8(12): 1514-24.