Journal of Peking University(Health Sciences) ›› 2017, Vol. 49 ›› Issue (5): 919-923. doi: 10.3969/j.issn.1671-167X.2017.05.032

• Article • Previous Articles     Next Articles

Renal Ewing’s sarcoma/primitive neuroectodermal tumor: a case report and literature review

LIU Chang1, CUI Li-gang1, WANG Hong-lei2   

  1. 1. Department of Ultrasound, Peking University Third Hospital, Beijing 100191, China;
    2. Department of Radiology, Peking University Third Hospital, Beijing 100191, China
  • Received:2015-12-29 Online:2017-10-18 Published:2017-10-18

Abstract: SUMMARY Ewing’s sarcoma/primitive neuroectodermal tumor (EWS/PNET) in the kidney is a rare but high-grade malignant tumor that affects predominantly elder children and adolescents. Patients mostly present with nonspecific symptoms such as abdominal pain and gross hematuria. Since EWS/PNET has a rapid clinical progression with early metastasis and death, it is essential to make an accurate and early diagnosis. Once diagnosed, multimodality treatment, including radical surgery combined with adjuvant chemotherapy, and radiotherapy if necessary, is recommended. Unfortunately, there are no characteristic signsthat have been described in ultrasonography or any other imaging modalities so far. The diagnosis of EWS/PNET is now based on a classical histological and immunohistochemical investigation complemented by a demonstration of specific chromosomal changes. Strong immunoreactivity to CD99 is ubiquitous, and t(11;22) translocation is seen in approximately 90% of EWS/PNET. Herein, we report a patient with such condition. The patient was a young woman, and she presented with sudden right flank pain clinically. Ultrasonography revealed a large heterogeneous mass in the lower pole of her right kidney. The tumor compressed the renal pelvis and led to upper pole caliectasis. Color Doppler demonstrated blood flow with a pulsatile arterialized waveform within the mass. The patient received radical nephrectomy with right renal vein and vena cava thrombectomy. A search for other sites of tumor involvement yielded negative results. And six cycles of chemotherapy were sequentially performed. The diagnosis of EWS/PNET was confirmed based on primitive small round cell histology and characteristic immunohistochemical results. She was still alive with no evidence of recurrence five years after initial diagnosis. We would like to point out that ultrasound is still a useful method for initial assessment, and ultrasound-guided fine needle aspiration may play an important role in determining preoperative diagnosis.

Key words: Sarcoma, Ewing’s, Neuroectodermal tumors, primitive, Kidney neoplasms, Immunohistochemistry, Ultrasonography

CLC Number: 

  • R737.11
[1] Almeida MF, Patnana M, Korivi BR, et al. Ewing sarcoma of the kidney: a rare entity [J]. Case Rep Radiol, 2014, 283902. doi:10.1155/2014/283902.
[2] Seemayer TA, Thelmo WL, Bolande RP, et al. Periphe-ral neuroectodermal tumors [J]. Perspect Pediatr Pathol, 1975, 2(2): 151-172.
[3] Pakravan A, Vo TM, Sandomirsky M, et al. Primary Ewing sarcoma of kidney in an elderly [J]. Iran J Kidney Dis, 2012, 6(4): 307-310.
[4] Jimenez RE, Folpe AL, Lapham RL, et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases [J]. Am J Surg Pathol, 2002, 26(3): 320-327.
[5] Karpate A, Menon S, Basak R, et al. Ewing sarcoma/primitive neuroectodermal tumor of the kidney: clinicopathologic analysis of 34 cases [J]. Ann Diagn Pathol, 2012, 16(4): 267-274.
[6] Rowe RG, Thomas DG, Schuetze SM, et al. Ewing sarcoma of the kidney: case series and literature review of an often overlooked entity in the diagnosis of primary renal tumors [J]. Urology, 2013, 81(2): 347-353.
[7] Zhang L, Wang T, Zheng L, et al. Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus [J]. J Natl Med Assoc, 2009, 101(12): 1291-1294.
[8] Hakky TS, Gonzalvo AA, Lockhart JL, et al. Primary Ewing sarcoma of the kidney: a symptomatic presentation and review of the literature [J]. Ther Adv Urol, 2013, 5(3): 153-159.
[9] Badar Q, Ali N, Abbasi N, et al. Ewing’s sarcoma/PNET of kidney in 13-year-old girl [J]. J Pak Med Assoc, 2010, 60(4): 314-315.
[10] Bing Z, Zhang P, Tomaszewski JE, et al. Primary Ewing sarcoma/primitive neuroectodermal tumor of the kidney [J]. J Urol, 2009, 181(3): 1341-1342.
[11] Parada D, Godoy A, Liuzzi F, et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney. An infrequent finding [J]. Arch Esp Urol, 2007, 60(3): 321-325.
[12] Saxena R, Sait S, Mhawech-Fauceglia P. Ewing sarcoma/primitive neuroectodermal tumor of the kidney: a case report. Diagnosed by immunohistochemistry and molecular analysis [J]. Ann Diagn Pathol, 2006, 10(6): 363-366.
[13] Lazzara BM, Scalcione LR, Garnet DJ, et al. Radiology-pathology conference: primary perinephric and renal extraosseous Ewing’s sarcoma [J]. Clin Imaging, 2012, 36(1): 77-79.
[14] Friedrichs N, Vorreuther R, Poremba C, et al. Primitive neuroectodermal tumor (PNET) in the differential diagnosis of malignant kidney tumors [J]. Pathol Res Pract, 2002, 198(8): 563-569.
[15] Kuroda M, Urano M, Abe M, et al. Primary primitive neuroectodermal tumor of the kidney [J]. Pathol Int, 2000, 50(12): 967-972.
[16] Risi E, Iacovelli R, Altavilla A, et al. Clinical and pa-thological features of primary neuroectodermal tumor/Ewing sarcoma of the kidney [J]. Urology, 2013, 82(2): 382-386.
[17] Vicha A, Stejskalova E, Sumerauer D, et al. Malignant peripheral primitive neuroectodermal tumor of the kidney [J]. Cancer Genet Cytogenet, 2002, 139(1): 67-70.
[18] Doerfler O, Reittner P, Groell R, et al. Peripheral pri-mitive neuroectodermal tumour of the kidney: CT findings [J]. Pediatr Radiol, 2001, 31(2): 117-119.
[19] Gupta NP, Singh BP, Raina V, et al. Primitive neuroectodermal kidney tumor: 2 case reports and review of the literature [J]. J Urol, 1995, 153(6): 1890-1892.
[20] Ellinger J, Bastian PJ, Hauser S, et al. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies [J]. Urology, 2006, 68(2): 257-262.
[21] Premalata CS, Gayathri Devi M, Biswas S, et al. Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology [J]. Acta Cytol, 2003, 47(3): 475-479.
[22] Kang SH, Perle MA, Nonaka D, et al. Primary Ewing sarcoma/PNET of the kidney: fine-needle aspiration, histology, and dual color break apart FISH Assay [J]. Diagn Cytopathol, 2007, 35(6): 353-357.
[23] Maly B, Maly A, Reinhartz T, et al. Primitive neuroectodermal tumor of the kidney. Report of a case initially diagnosed by fine needle aspiration cytology [J]. Acta Cytol, 2004, 48(2): 264-268.
[24] Kumar R, Gautam U, Srinivasan R, et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney: Report of a case diagnosed by fine needle aspiration cytology and confirmed by immunocytochemistry and RT-PCR along with review of literature [J]. Diagn Cytopathol, 2012, 40(suppl 2): e156-161.
[25] Sanati S, Lu DW, Schmidt E, et al. Cytologic diagnosis of Ewing sarcoma/peripheral neuroectodermal tumor with paired prospective molecular genetic analysis [J]. Can-cer, 2007, 111(3): 192-199.
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