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Year : 2018  |  Volume : 14  |  Issue : 6  |  Page : 1422-1424

Ewing's sarcoma of kidney in a 60-year-old patient with local recurrence: A rare occurrence

1 Department of Medical Oncology, Gazi University Faculty of Medicine, Ankara, Turkey
2 Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey

Date of Web Publication28-Nov-2018

Correspondence Address:
Irem Bilgetekin
Department of Medical Oncology, Gazi University Faculty of Medicine, Besevler, Ankara
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.191062

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 > Abstract 

Ewing's family of tumors is aggressive tumors and frequently arises from bone and soft tissue. They might also arise from nonosseous structures such as gastrointestinal tract, adrenal glands, or kidney. Primary renal Ewing's sarcoma (ES)/primitive neuroectodermal tumor is an extremely rare entity which has aggressive clinical course. These high-grade malignant tumors predominantly affect adolescents and young adults. Patients mostly present with nonspecific symptoms such as pain, hematuria, mass, and sensitivity. It is confused with renal cell cancer in imaging techniques. The definitive diagnosis is based on the histopathological examination. Surgical or radiotherapy treatment is used for local control and multiagent chemotherapy used for systemic treatment. Despite all treatment options, prognosis is poor. We aimed to describe the diagnosis and follow-up and treatment of renal ES case that was considered as renal cell carcinoma in imaging but diagnosed as ES via histopathology.

Keywords: Diagnosis, histopathology, renal cell carcinoma, renal Ewing sarcoma, treatment

How to cite this article:
Bilgetekin I, Karaca M, Gönül II, Üner A, Şahinli H, Demir H, Aytekin A, Çiltaû A, Benekli M. Ewing's sarcoma of kidney in a 60-year-old patient with local recurrence: A rare occurrence. J Can Res Ther 2018;14:1422-4

How to cite this URL:
Bilgetekin I, Karaca M, Gönül II, Üner A, Şahinli H, Demir H, Aytekin A, Çiltaû A, Benekli M. Ewing's sarcoma of kidney in a 60-year-old patient with local recurrence: A rare occurrence. J Can Res Ther [serial online] 2018 [cited 2021 Dec 1];14:1422-4. Available from: https://www.cancerjournal.net/text.asp?2018/14/6/1422/191062

 > Introduction Top

Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are common in childhood and mainly occur in the bone or soft tissues of the extremities such as humerus, femur, and tibia and less commonly in the viscera or kidneys.[1] Ewing's family of tumors (ESFT) consists of Ewing's sarcoma, extraosseous sarcomas, and PNET which shares common stem-cell precursor and unique chromosomal abnormality. These tumors are high grade and they are from a family that is called as small, round blue cell tumors. Extraskeletal ESFTs are less common and can affect the skin, soft tissue, or viscera.[2] ES that originates from kidney is an extremely rare entity, which has been documented throughout the literature as isolated case reports to date.[3] We aimed to describe the aspects of diagnosis and treatment of renal ES in a patient with imaging and pathological analysis.

 > Case Report Top

A 61-year-old male patient presented to our center with complaints of 2 months of left lumbar pain that was nonradiating and dull in character. Only costovertebral angle tenderness was present in physical examination. Ultrasonography showed an 8 cm × 7 cm in size exophytic cortical cyst with irregular echogenic septation in the centrum in the left kidney. Abdominal magnetic resonance imaging had partial opacity in the left kidney and that was reported as first hemorrhagic papillary renal cell carcinoma (RCC) should be considered. In abdominal computed tomography (CT) in anterior region of the upper middle part of the left kidney, a 10 cm × 7 cm × 9 cm RCC mass that has exophytic growth and areas of necrosis was observed. The image was found to be compatible with renal cell cancer. Left radical nephrectomy was performed with the diagnosis of left renal tumor, and the pathology result was reported as ES/PNET. In immunohistochemical examination that was performed in terms of the primary tumor, CD45, CD43, and CD20 were negative for hematolymphoid malignancy, and the cyclin D-1 diffuse positive cells were found. The cells were positive for the PNET markers: Diffuse uniform membranous staining was present with CD99. CD117 was cytoplasmic and membranous positive [Figure 1]a, [Figure 1]b, [Figure 1]c. Further characterization was accomplished by cytogenetic studies, Ewing's sarcoma/Friend leukemia integration 1 (EWS/FLI-1) broke apart working with in situ hybridization probes, and EWS/FLI-1 translocation was determined. The features were consistent with a primary renal PNET. No metastasis was found. Our patient was treated by eight cycles of chemotherapy every 3 weeks including vincristine, cyclophosphamide, adriamycin, ifosfamide, mesna, and etoposide. Metastasis was identified in liver, left nephrectomy region, hepatorenal fossa, and left psoas muscle in follow-up of 6 months. Then, gemcitabine and docetaxel were administered. After three cycles of chemotherapy, control CT was performed and mass lesion was observed reaching about 20 cm size in muscular structure in the left kidney region [Figure 2]. The patient that was followed due to intra-abdominal sepsis died owing to septic shock.
Figure 1: (a) Solid mass with geographic necrosis located in the renal hilum (H and E, ×40), (b) small blue round cells with almost uniform morphology in Ewing's sarcoma/primitive neuroectodermal tumor (H and E, ×400), (c) membranous CD99 positivity in the neoplastic cells, streptavidin-biotin peroxidase (×400)

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Figure 2: Representative axial computed tomography images of patient with a large multilobulated mass in the interpolar region of the left kidney

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 > Discussion Top

The small, round cell tumors of the kidney include a wide range of unrelated neoplasms with overlapping histomorphology and they have different prognostic/therapeutic characteristics. The tumors usually incorporated in this differential diagnosis include blastema predominant Wilms' tumor, ES/PNET, metastatic neuroblastoma, monophasic synovial sarcoma, desmoplastic round cell tumor, small cell neuroendocrine carcinoma, clear cell sarcoma of kidney, alveolar/embryonal rhabdomyosarcoma, sarcomatous dedifferentiated RCC, and non-Hodgkin's lymphoma. This differential diagnosis is further complicated by the relatively rare occurrence of most of these entities in the kidney. Although RCC is a common malignancy of the kidney, primary renal sarcomas occur quietly rarely and exhibit highly aggressive biological behavior.[4]

Renal ES/PNET was first described by Seemayer et al. in 1975.[5] The average age of occurrence is between 28 and 34 years of age and it is seen more frequent in males.[4],[6] Our patient was 61 years, which is an age at which it occurs rarely.

The clinical symptoms and signs are not characteristic and the patient frequently presents with acute flank pain mimicking renal stone colic (73%), renal mass on imaging studies, hematuria, and sensitivity in decreasing order.[5] In renal ES, imaging techniques do not show clear signs[6] and as in our case who was first considered as RCC. ES is definitively diagnosed by histopathology with a detailed immunohistochemical analysis. In pathological image, small round blue cells that involve necrotic areas are observed. Diffuse uniform membranous staining with CD99, vimentin, and CD117 may be detected in cells.[7] Furthermore, in our case, the CD99 and CD117 positivity was detected. However, the gold standard of the diagnosis was the identification of the same EWS/FLI-1 gene fusions in renal ES/PNET as in extrarenal tumors.[8] While diagnosis may not be established by fluorescent in situ hybridization, which is important to support the accurate diagnosis, EWS/FLI-1 fusion is used for confirmation.

Surgical resection in the treatment of disease is an important method for local control. However ES may completely be resectable, rapidly recur, or have metastasis. Therefore, systemic chemotherapy for yielding local control (surgery or radiotherapy or these modalities in combination) may be used. Despite aggressive treatment, the median survival was 26.14 months in all stages and 5.6 months in advanced metastatic stage.[9] Although no randomized studies have been published in the setting of the rarity of renal ES/PNET, ifosfamide-based chemotherapy can provide a positive impact on overall survival.[10] Most of the multiagent chemotherapy regimens that extrapolated from the treatment regimens for osseous ES, consisted of multi-drugs may be given. However, different responses to chemotherapy regimens are observed. The most potent agents involve vincristine, actinomycin D, high-dose cyclophosphamide, doxorubicin, etoposide, and ifosfamide combinations. Further, in our case, we carried out radical surgery followed by combination chemotherapy regimens, but disease still progressed.

 > Conclusion Top

The definitive diagnosis of ES that may not be differentiated from RCC via imaging techniques can be made using histopathology.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Balamuth NJ, Womer RB. Ewing's sarcoma. Lancet Oncol 2010;11:184-92.  Back to cited text no. 1
Wedde TB, Lobmaier IV, Brennhovd B, Lohne F, Hall KS. Primary Ewing's sarcoma of the kidney in a 73-year-old man. Sarcoma 2011;2011:978319.  Back to cited text no. 2
Lalwani N, Prasad SR, Vikram R, Katabathina V, Shanbhogue A, Restrepo C. Pediatric and adult primary sarcomas of the kidney: A cross-sectional imaging review. Acta Radiol 2011;52:448-57.  Back to cited text no. 3
Angel JR, Alfred A, Sakhuja A, Sells RE, Zechlinski JJ. Ewing's sarcoma of the kidney. Int J Clin Oncol 2010;15:314-8.  Back to cited text no. 4
Seemayer TA, Thelmo WL, Bolande RP, Wiglesworth FW. Peripheral neuroectodermal tumors. Perspect Pediatr Pathol 1975;2:151-72.  Back to cited text no. 5
Ekram T, Elsayes KM, Cohan RH, Francis IR. Computed tomography and magnetic resonance features of renal Ewing sarcoma. Acta Radiol 2008;49:1085-90.  Back to cited text no. 6
Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, et al. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: A clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol 2002;26:320-7.  Back to cited text no. 7
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.  Back to cited text no. 8
Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, et al. Primary disseminated multifocal Ewing sarcoma: Results of the Euro-EWING 99 trial. J Clin Oncol 2010;28:3284-91.  Back to cited text no. 9
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.  Back to cited text no. 10


  [Figure 1], [Figure 2]

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