Ewing’s Sarcoma with Extension into Superior Vena Cava and Right Atrium

  • Yukari AtsumiEmail author
  • Yuya Saito
  • Hiroshi Hataya
  • Ryuji Fukuzawa
  • Yuki Yuza
Case Report


Ewing sarcoma is rarely shown to develop this intravascular extension so the decision of the initial treatment is more difficult. We report a 7-year-old boy of this sarcoma with extension into superior vena cava (SVC) and right atrium (RA), who was successfully treated with initial surgery. Intravascular extension was observed from the azygous vein to SVC and finally RA. The removal of the intravascular extension was done, 7 days before chemotherapy was started. The initial surgery for the intravascular extension may have decreased a risk of pulmonary tumor embolism and this made the chemotherapy done safe in this patient.


Ewing’s sarcoma Intravascular extension Tumor embolism Initial treatment 



We would like to thank the members of the department of cardiovascular surgery for their participation in the operation and we also thank Mr. James R. Valera for English proofreading.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.


  1. 1.
    Abdullah Y, Karpelowsky J, Davidson A, Thomas J, Brooks A, Hewitson J, Numanoglu A, Cox S, Millar AJW (2013) Management of nine cases of Wilms’ tumour with intracardiac extension - a single centre experience. J Pediatr Surg 48:394–399CrossRefGoogle Scholar
  2. 2.
    Vaideeswar P, Chaudhari JP (2012) Wilms’ tumor with right heart extension: report of a post-chemotherapeutic fatality. Indian J Pathol Microbiol 55:381–383CrossRefGoogle Scholar
  3. 3.
    Le Deley MC, Paulussen M, Lewis I, Brennan B, Ranft A, Whelan J, Le Teuff G, Michon J, Ladenstein R, Marec-Berard P, van den Berg H, Hjorth L, Wheatley K, Judson I, Juergens H, Craft A, Oberlin O, Dirksen U (2014) Cyclophosphamide compared with ifosfamide in consolidation treatment of standard-risk Ewing sarcoma: results of the randomized noninferiority Euro-EWING99-R1 trial. Journal of clinical oncology : official journal of the American Society of Clinical Oncology 32:2440–2448CrossRefGoogle Scholar
  4. 4.
    Varga-Szemes A, Schoepf UJ, Spruill LS, Suranyi PA (2015) Rare case of intrapulmonary Ewing sarcoma presenting with left atrial tumor thrombus. Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer 10:1120–1122CrossRefGoogle Scholar
  5. 5.
    Mete UK, Singh DV, Bhattacharya A, Kakkar N (2015) Fulminant Buddchiari syndrome caused by renal primiive neuroectodermal tumor with inferior vena cava thrombus extending to atrium. J Cancer Res Ther 11:1038PubMedGoogle Scholar
  6. 6.
    Saboo SS, Krajewski KM, Jagannathan JP, Ramaiya N (2012) IVC tumor thrombus: an advanced case of rare extraosseous Ewing sarcoma of the adrenal gland. Urology 79:e77–e78CrossRefGoogle Scholar
  7. 7.
    Wang J, Wang W, Li Y, Miao Y, Liu W, Yao S, Liao Y, Ouyang C (2015) A primary primitive neuroectodermal tumor arising from left subclavian vein and extending along left brachiocephalic vein and superior vena cava into right atrium. Ann Vasc Surg 29:839.e13–839.e20CrossRefGoogle Scholar
  8. 8.
    Murphy SM, Browne RF, Finn S, Myers E, Crotty P, Grainger R (2003) Non-metastatic primitive peripheral neuroectodermal tumour of the kidney (extraskeletal Ewing’s sarcoma) with vena caval tumour thrombus. BJU Int 92(Suppl 3):e44PubMedGoogle Scholar
  9. 9.
    Fergany AF, Dhar N, Budd GT, Skacel M, Garcia JA (2009) Primary extraosseous Ewing sarcoma of the kidney with level III inferior vena cava thrombus. Clin Genitourin Cancer 7:E95–E97CrossRefGoogle Scholar
  10. 10.
    Funakoshi Y, Mukohara T, Kataoka T et al (2010) Left atrial extension of metastatic lung tumor via pulmonary vein: report on the first case of Ewing sarcoma. Rare Tumors 2(3):53CrossRefGoogle Scholar
  11. 11.
    Alonso AH, Garate MM, Amo FH et al (2011) Primary renal Ewing's sarcoma. Arch Esp Urol 64:636–639PubMedGoogle Scholar
  12. 12.
    Cho SI, Park YH, Cho JH, Ryoo BY, Yang SH, Youn SM, Ko JS (2007) Extraskeletal Ewing’s sarcoma of the head and neck presenting as blindness. Korean J Intern Med 22:133–137CrossRefGoogle Scholar
  13. 13.
    McMahon S, Carachi R (2014) Wilms’ tumor with intravascular extension: a review article. J Indian Assoc Pediatr Surg 19:195–200CrossRefGoogle Scholar

Copyright information

© Indian Association of Surgical Oncology 2019

Authors and Affiliations

  • Yukari Atsumi
    • 1
    Email author
  • Yuya Saito
    • 2
  • Hiroshi Hataya
    • 1
  • Ryuji Fukuzawa
    • 3
  • Yuki Yuza
    • 2
  1. 1.Department of General PediatricsTokyo Metropolitan Children’s Medical CenterTokyoJapan
  2. 2.Department of Hematology and OncologyTokyo Metropolitan Children’s Medical CenterTokyoJapan
  3. 3.Department of PathologyTokyo Metropolitan Children’s Medical CenterTokyoJapan

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