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Kidney Ewing Sarcoma

MedGen UID:
1702234
•Concept ID:
C5204258
•
Neoplastic Process
Synonym: Renal Ewing Sarcoma

Definition

A rare Ewing sarcoma arising from the kidney. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKidney Ewing Sarcoma

Professional guidelines

PubMed

Guide for paediatric radiotherapy procedures.
Laprie A, Bernier V, Padovani L, Martin V, Chargari C, Supiot S, Claude L
Cancer Radiother 2022 Feb-Apr;26(1-2):356-367. Epub 2021 Dec 27 doi: 10.1016/j.canrad.2021.11.018. PMID: 34969622
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2014 Sep 4;(9):CD006647. doi: 10.1002/14651858.CD006647.pub4. PMID: 25188452
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2011 Jan 19;(1):CD006647. doi: 10.1002/14651858.CD006647.pub3. PMID: 21249679

Recent clinical studies

Etiology

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents.
Wallace MW, Niec JA, Ghani MOA, McKay KG, Idrees K, Liang J, Borinstein SC, Lovvorn HN 3rd
J Pediatr Surg 2023 Sep;58(9):1727-1735. Epub 2023 Jan 14 doi: 10.1016/j.jpedsurg.2023.01.006. PMID: 36774201
The 2022 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.
Moch H, Amin MB, Berney DM, Compérat EM, Gill AJ, Hartmann A, Menon S, Raspollini MR, Rubin MA, Srigley JR, Hoon Tan P, Tickoo SK, Tsuzuki T, Turajlic S, Cree I, Netto GJ
Eur Urol 2022 Nov;82(5):458-468. Epub 2022 Jul 16 doi: 10.1016/j.eururo.2022.06.016. PMID: 35853783
Modern Therapy for Spinal and Paraspinal Ewing Sarcoma: An Update of the University of Florida Experience.
Indelicato DJ, Vega RBM, Viviers E, Morris CG, Bradfield SM, Ranalli NJ, Bradley JA
Int J Radiat Oncol Biol Phys 2022 May 1;113(1):161-165. Epub 2022 Jan 13 doi: 10.1016/j.ijrobp.2022.01.007. PMID: 35033584
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Ewing sarcoma of the kidney: case series and literature review of an often overlooked entity in the diagnosis of primary renal tumors.
Rowe RG, Thomas DG, Schuetze SM, Hafez KS, Lawlor ER, Chugh R
Urology 2013 Feb;81(2):347-53. doi: 10.1016/j.urology.2012.10.016. PMID: 23374800

Diagnosis

The 2022 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.
Moch H, Amin MB, Berney DM, Compérat EM, Gill AJ, Hartmann A, Menon S, Raspollini MR, Rubin MA, Srigley JR, Hoon Tan P, Tickoo SK, Tsuzuki T, Turajlic S, Cree I, Netto GJ
Eur Urol 2022 Nov;82(5):458-468. Epub 2022 Jul 16 doi: 10.1016/j.eururo.2022.06.016. PMID: 35853783
Primary Ewing sarcoma of the kidney: A series of four cases.
Patra S, Trivedi P
Malays J Pathol 2022 Apr;44(1):93-99. PMID: 35484891
Renal Ewing Sarcoma: A Challenging Diagnosis.
Chew FY, Wu SH, Lin WC
Iran J Kidney Dis 2021 Sep;15(5):327. PMID: 34582366
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687

Therapy

Modern Therapy for Spinal and Paraspinal Ewing Sarcoma: An Update of the University of Florida Experience.
Indelicato DJ, Vega RBM, Viviers E, Morris CG, Bradfield SM, Ranalli NJ, Bradley JA
Int J Radiat Oncol Biol Phys 2022 May 1;113(1):161-165. Epub 2022 Jan 13 doi: 10.1016/j.ijrobp.2022.01.007. PMID: 35033584
A rare entity of Primary Ewing sarcoma in kidney.
Cheng L, Xu Y, Song H, Huang H, Zhuo D
BMC Surg 2020 Nov 11;20(1):280. doi: 10.1186/s12893-020-00948-9. PMID: 33176766Free PMC Article
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687
Ewing sarcoma of the kidney: case series and literature review of an often overlooked entity in the diagnosis of primary renal tumors.
Rowe RG, Thomas DG, Schuetze SM, Hafez KS, Lawlor ER, Chugh R
Urology 2013 Feb;81(2):347-53. doi: 10.1016/j.urology.2012.10.016. PMID: 23374800
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2011 Jan 19;(1):CD006647. doi: 10.1002/14651858.CD006647.pub3. PMID: 21249679

Prognosis

Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations.
Whittle SB, Fetzko S, Roy A, Venkatramani R
J Pediatr Hematol Oncol 2022 Jul 1;44(5):195-200. Epub 2022 May 4 doi: 10.1097/MPH.0000000000002480. PMID: 35537005Free PMC Article
Primary Ewing sarcoma of the kidney: A series of four cases.
Patra S, Trivedi P
Malays J Pathol 2022 Apr;44(1):93-99. PMID: 35484891
The Role of FDG-PET and Whole-Body MRI in High Grade Bone Sarcomas With Particular Focus on Osteosarcoma.
Farina A, Gasperini C, Aparisi Gómez MP, Bazzocchi A, Fanti S, Nanni C
Semin Nucl Med 2022 Sep;52(5):635-646. Epub 2021 Dec 6 doi: 10.1053/j.semnuclmed.2021.10.001. PMID: 34879906
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.
Celli R, Cai G
Arch Pathol Lab Med 2016 Mar;140(3):281-5. doi: 10.5858/arpa.2014-0367-RS. PMID: 26927724

Clinical prediction guides

Late toxicity comparison of alkylating-based maintenance regimen with cyclophosphamide (VAC) vs ifosfamide (VAI) in Ewing sarcoma survivors treated in the randomized clinical trial Euro-EWING99-R1 in France.
Corvest V, Marec-Bérard P, Lervat C, Pacquement H, Toulmonde M, Gentet JC, Laurence V, Cleirec M, Mansuy L, Bompas E, Castex MP, Taque S, Filhon B, Tabone MD, Verité C, Entz-Werle N, Saumet L, Guimard G, Pondrom M, Chevreau C, Flandrin J, Duranteau L, Rousset-Jablonski C, Brugières L, Jimenez M, Le Deley MC, Gaspar N, Fresneau B
Int J Cancer 2023 Apr 15;152(8):1659-1667. Epub 2022 Oct 31 doi: 10.1002/ijc.34326. PMID: 36250317Free PMC Article
The Role of FDG-PET and Whole-Body MRI in High Grade Bone Sarcomas With Particular Focus on Osteosarcoma.
Farina A, Gasperini C, Aparisi Gómez MP, Bazzocchi A, Fanti S, Nanni C
Semin Nucl Med 2022 Sep;52(5):635-646. Epub 2021 Dec 6 doi: 10.1053/j.semnuclmed.2021.10.001. PMID: 34879906
Primary round cell sarcomas of the urinary bladder with EWSR1 rearrangement: a multi-institutional study of thirteen cases with a review of the literature.
Baisakh MR, Tiwari A, Gandhi JS, Naik S, Sharma SK, Balzer BL, Sharma S, Peddinti K, Jha S, Sahu PK, Pradhan D, Geller M, Amin MB, Dhillon J, Mohanty SK
Hum Pathol 2020 Oct;104:84-95. Epub 2020 Aug 14 doi: 10.1016/j.humpath.2020.08.001. PMID: 32798549
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Renal Ewing tumors.
Zöllner S, Dirksen U, Jürgens H, Ranft A
Ann Oncol 2013 Sep;24(9):2455-61. Epub 2013 Jun 11 doi: 10.1093/annonc/mdt215. PMID: 23761687

Recent systematic reviews

Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2014 Sep 4;(9):CD006647. doi: 10.1002/14651858.CD006647.pub4. PMID: 25188452
Systematic review of ablation techniques for the treatment of malignant or aggressive benign lesions in children.
Gómez FM, Patel PA, Stuart S, Roebuck DJ
Pediatr Radiol 2014 Oct;44(10):1281-9. Epub 2014 May 13 doi: 10.1007/s00247-014-3001-5. PMID: 24821394
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2011 Jan 19;(1):CD006647. doi: 10.1002/14651858.CD006647.pub3. PMID: 21249679
Treatment including anthracyclines versus treatment not including anthracyclines for childhood cancer.
van Dalen EC, Raphaël MF, Caron HN, Kremer LC
Cochrane Database Syst Rev 2009 Jan 21;(1):CD006647. doi: 10.1002/14651858.CD006647.pub2. PMID: 19160293

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