Primary Ewing sarcoma of the kidney: a symptomatic presentation and review of the literature

Hakky, Tariq S.; Gonzalvo, Americo; Lockhart, Jorge L.; Rodríguez, Alejandro

doi:10.1177/1756287212471095

Cited by 46 publications

(52 citation statements)

References 20 publications

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“…Other authors theorize that embryonic neural cells migrate into the kidney and undergo tumorigenesis [1]. Hakky et al [9] in their case report and meta-analysis have described acute flank pain mimicking renal colic as the most common presentation of EWS of the kidney. Our patient also presented with flank pain of short duration which supports their finding.…”

Section: Discussionmentioning

confidence: 99%

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Ewings Sarcoma of the Kidney: A Rare Entity

Choubey

Pipara

Kumar

2017

World J Nephrol Urol

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Primary Ewings sarcoma (EWS) of the kidney is a very rare tumor and very few cases have been reported till date. We present a case of a young girl presenting to us with flank pain. On evaluation, she was found to have a renal mass. She was explored and a nephrectomy was done. Histopathology was reported as EWS. She was then planned for adjuvant chemotherapy. Primary EWS of the kidney is characterized by an aggressive course and poor prognosis. Hence, while dealing with renal masses in young patients, this entity should always be kept in mind.

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Section: Discussionmentioning

confidence: 99%

“…It was first described in 1975 by Seemayer and colleagues [1]. Only very few cases of primary EWS have been reported in the literature till date [2][3][4][5][6][7][8][9]. Sources of renal EWS include neural cells that invaginate into the kidney during development.…”

Section: Introductionmentioning

confidence: 99%

Ewings Sarcoma of the Kidney: A Rare Entity

Choubey

Pipara

Kumar

2017

World J Nephrol Urol

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“…4 In our case, the patient initially underwent radical nephrectomy because of the high likelihood of RCC. The final diagnosis of ES was confirmed through immunohistochemical analysis for cytokeratin, vimentin, and CD99.…”

Section: Discussionmentioning

confidence: 85%

“…1 However, Hakky and colleagues discovered that renal ES commonly presents with symptoms, while RCC is an incidental finding on imaging. 4 In our case, the patient initially underwent radical nephrectomy because of the high likelihood of RCC. The …”

Section: Discussionmentioning

confidence: 85%

Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma

Kim

Chae

Yoon

et al. 2015

CUAJ

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We recently encountered an extremely rare case of spontaneous perirenal hemorrhage in a 34-year-old man. He initially had undergone radical nephrectomy owing to suspicion of renal cell carcinoma. The final diagnosis was extraskeletal Ewing sarcoma. IntroductionEwing sarcoma (ES) is a poorly differentiated, aggressive malignancy that most frequently develops in bone, soft tissues, and the chest wall. Although the kidney has been increasingly recognized as an unexpected site for extraskeletal ES, 1 primary renal ES is still uncommon, furthermore, spontaneous rupture of primary renal ES is rarely reported. We present an extremely rare case of spontaneous perirenal hemorrhage because of renal ES. Case reportA 34-year-old man presented to the emergency department with left flank pain for 1 day. He had no history of specific disease or trauma. Physical examination indicated moderate tenderness across the whole abdomen, which was more severe on the left side. No rebound tenderness was noted in the abdomen. However, severe costovertebral angle tenderness was present on the left side.He had a blood pressure of 140/90 mmHg, pulse rate of 95 beats/min, respiratory rate of 20 breaths/min, and body temperature of 37.7°C. A complete blood count was performed, the results of which revealed a hemoglobin of 14.3 g/ dL, a platelet count of 176 000/µL, a blood urea nitrogen level of 18.5 mg/dL, and a creatinine level of 0.92 mg/dL.An urgent contrast-enhanced computed tomography (CT) scan was performed (Fig. 1). We visualized a suspicious 6.8-cm mass originating from the upper pole of the left kidney, with internal necrosis and hemorrhage in the left perirenal space. The first impression of the radiologist was spontaneous rupture of renal cell carcinoma (RCC), and the second was fat depletion angiomyolipoma. On the third day of hospitalization, we performed a radical nephrectomy due to potential of malignancy.Macroscopic examination revealed a ruptured tumour mass with circumjacent hemorrhage, and the ruptured surface showed hemorrhagic and adipose tissue. Microscopically, the tumours were in the renal pelvis and ureter, infiltrating to the renal parenchyma (Fig. 2a). They consisted of uniform, small round cells with round nuclei containing fine chromatin, scanty clear, or eosinophilic cytoplasm, and indistinct cytoplasmic membranes (Fig. 2b). In the immunohistochemical analysis, the cells exhibited diffuse membranous positive staining with CD99 and vimentin, while cytokeratin was negative (Fig. 2c). The EWS-FLI1 fusion gene consistent with t(11;22) demonstrated by fluorescence in situ hybridization study (Fig. 2d). The pathologic diagnosis was high-grade extraskeletal ES.In addition, we performed a chest CT scan, whole body positron emission tomography (PET)-CT scan, and whole body bone scan that revealed no other pathologic site (primary origin). The final diagnosis was spontaneous perirenal hemorrhage of primary renal ES. He was discharged 10 days after nephrectomy, and received adjuvant chemotherapy (3 cycles of vincris...

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“…Based on the aforementioned studies, we suggest radiotherapy in the presence of residual disease after surgical dissection, positive surgical margins, and loco-regional lymphadenopathy. Although it is speculated that radiotherapy should be avoided as the first-line treatment; it may prove beneficial in salvage setting [13].…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing sarcoma of the kidney: a case report and treatment review

et al. 2017

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Ewing sarcomas/primitive neuroectodermal tumors (ES/PNET) of the kidney are rarely found high-grade malignant tumors, offering poor prognosis. Although established treatment guidelines for ES of kidney are scarce, a multi-modality treatment approached is typically implemented. Herein, we report a 14-year-old female patient with ES of right kidney. Post-nephrectomy disease recurrence was treated with chemotherapy (i.e., vincristine, doxorubicin and cyclophosphamide); marked reduction in tumor size (i.e., from 18.5 9 11.3 cm 2 to 3.7 9 2.2 cm 2 ; *96% reduction in size) as per computed tomography images was observed. We present our treatment experience and review from the available literature.

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Primary Ewing sarcoma of the kidney: a symptomatic presentation and review of the literature

Cited by 46 publications

References 20 publications

Ewings Sarcoma of the Kidney: A Rare Entity

Ewings Sarcoma of the Kidney: A Rare Entity

Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma

Primary Ewing sarcoma of the kidney: a case report and treatment review

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