A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing's sarcoma of the kidney with renal vein tumor thrombus

Athens Medical Center, Athínai, Attica, Greece
International Urology and Nephrology (Impact Factor: 1.52). 02/2007; 39(2):393-5. DOI: 10.1007/s11255-006-9073-x
Source: PubMed


Primitive neuroectodermal tumor/extraosseous Ewing's sarcoma (PNET/EES) is a childhood disease rarely seen in adults. It is a soft tissue tumor, which is often observed in the paraspinal region and lower extremity. We report the case of a 32-year-old man who presented with sudden abdominal pain on the right upper quadrant that was radiated to the right flank. During the operation, a spontaneously ruptured right kidney mass was found. The histopathologic and immunohistochemical characteristics of the excised mass were consistent with PNET/EES. This is the first known reported case of spontaneously ruptured PNET/EES of the kidney with renal vein tumor thrombus. The clinical details and the management of this case are described.

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    • "As far as literature reports, there are few reports of renal primitive neuroectodermal tumor/EWING with tumor thrombus extending to the inferior vena cava [9, 10]. However, in our case the thrombus extended all the way up to the inferior vena cava, right atrium, and bilateral pulmonary arteries, with more aggressive tumor extension than the previous reported cases [9, 10]. "
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    ABSTRACT: Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this paper illustrated the difficulties with which routine anatomical pathologist is faced when dealing with rare renal poorly differentiated neoplasm in adults.
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