Böbreğin primitif nöroektodermal tümörü / Ewing sarkomu (pnet/ews): Olgu sunumu
Sol yan ağrısı ile başvuran 44 yaşındaki bir vaka sunuldu. Bilgisayarlı karın tomografisinde sol böbrek üst polünde yer alan renal kitle saptandı. Hastaya sol radikal nefrektomi ve adrenalektomi yapıldı. Cerrahi örneğin patolojik inceleme sonucunda böbreğin primitif nöroektodermal tümör / Ewing sarkomu saptandı. Nispeten nadir olan bu tümörün klinik bulguları, görüntüleme bulguları ve tanısı tartışıldı
Primitive neuroectodermal tumor / Ewing sarcoma (pnet/ews) of the kidney: Report of a case
The case of a 44-year-old man who presented with left flank pain is reported. Computer tomography of the abdomen revealed a left renal mass replacing upper pole of the kidney. The patient underwent left radical nephrectomy and adrenalectomy. Pathologic examination of the surgical specimen revealed the primitive neuroectodermal tumour / Ewing sarcoma of the kidney. The clinical presentation, imaging findings and diagnosis of this relatively rare tumor are discussed.
___
- 1. Toskos M. Peripheral primitive neuroectodermal tumors: Diagnosis, classification and prognosis. Perspect Pediatr Pathol 1992;16:27-98.
- 2. Horowitz ME, Delaney TF, Malawer MM, Woo SY, Hicks MJ. Ewing’s sarcoma of bone and soft tissue and peripheral primitive neuroectodermal tumor. In: Pizzo PA, Poplack PG, editors. Principles and practice of pediatric oncology. Philadelphia7 Lippincott; 1997. p.831- 63.
- 3. Parham DM, Roloson GJ, Feely M, Green DM, Bridge JA, Beckwith JB. 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;25:133-46.
- 4. Ellinger J, Bastian PJ, Hauser S, Biermann K, Müller SC. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology 2006;68:257-62.
- 5. McKeon C, Thiele CJ, Ross RA, Kwan M, Triche TJ, Miser JS, Israel MA. Indistinguishable patterns of protooncogene expression in two distinct but closely related tumors: Ewing’s sarcoma and neuroepithelioma. Cancer Res 1988;48:4307- 11.
- 6. Risi E, Iacovelli R, Altavilla A, Alesini D, Palazzo A, Mosillo C, et al. Clinical and pathological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney. Urology 2013;82:382-386.
- 7. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, 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;348:694-701.