Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

  • Tao, Hai-Tao (Department of Oncology, China PLA General Hospital) ;
  • Hu, Yi (Department of Oncology, China PLA General Hospital) ;
  • Wang, Jin-Liang (Department of Oncology, China PLA General Hospital) ;
  • Cheng, Yao (Department of Oncology, China PLA General Hospital) ;
  • Zhang, Xin (Department of Oncology, China PLA General Hospital) ;
  • Wang, Huan (Department of Oncology, China PLA General Hospital) ;
  • Zhang, Su-Jie (Department of Oncology, China PLA General Hospital)
  • Published : 2013.05.30


Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.


  1. Ahmad R, Mayol BR, Davis M, et al (1999). Extraskeletal Ewing's sarcoma. Cancer, 85, 725-31.<725::AID-CNCR23>3.0.CO;2-2
  2. Applebaum MA, Worch J, Matthay KK, et al (2011). Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer, 117, 3027-32.
  3. Balamuth NJ, Womer RB (2010). Ewing's sarcoma. Lancet Oncol, 11, 184-92.
  4. Baldini EH, Demetri GD, Fletcher CD, et al (1999). Adults with Ewing's sarcoma/primitive neuroectodermal tumour: adverse effect of older age and primary extraosseous disease on outcome. Ann Surg, 230, 79-86.
  5. Downing JR, Head DR, Parham DM, et al (1993). Detection of the (11; 22) (q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumour by reverse transcription polymerase chain reaction. Am J Pathol, 143, 1294-300.
  6. Dunst J, Schuck A (2004). Role of radiotherapy in Ewing tumours. Pediatr Blood Cancer, 42, 465-70.
  7. El Weshi A, Allam A, Ajarim D, et al (2010). Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institute. Clin Oncol (R Coll Radiol), 22, 374-81.
  8. El Weshi A, Memon M, Raja M, et al (2004). VIP (etoposide, ifosfamide, cisplatin) in adult patients with recurrent or refractory Ewing sarcoma family of tumours. Am J Clin Oncol, 27, 529-34.
  9. Eralp Y, Bavbek S, Basaran M, et al (2002). Prognostic factors and survival in late adolescent and adult patients with small round cell tumours. Am J Clin Oncol, 25, 418-24.
  10. Grier HE, Krailo MD, Tarbell NJ, et al (2003). Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumour of bone. N Engl J Med, 348, 694-701
  11. Gururangan S, Marina NM, Luo X, et al (1998). Treatment of children with peripheral primitive neuroectodermal tumour or extraosseous Ewing's tumour with Ewing's-directed therapy. J Pediatr Hematol Onco, 20, 55-61.
  12. Herzog CE (2005). Overview of sarcomas in the adolescent and young adult population. J Pediatr Hematol Oncol, 27, 215-8.
  13. Ibrahim N, Abou-Elela E, Darwish D (2013). Survival of mesothelioma in a palliative medical care unit in egypt. Asian Pac J Cancer Prev, 14, 739-42.
  14. Kaspers GJ, Kamphorst W, van de Graaff M, et al (1991). Primary spinal epidural extraosseous Ewing's sarcoma. Cancer, 68, 648-54.<648::AID-CNCR2820680335>3.0.CO;2-Q
  15. Kinsella TJ, Triche TJ, Dickman PS, et al (1983). Extraskeletal Ewing's sarcoma: results of combined modality treatment. J Clin Oncol, 1, 489-95.
  16. Lasrado S, Prabhu P, Kakria A, et al (2012), Clinicopathological profile of head and neck cancers in the Western development region, Nepal: a 4-year snapshot. Asian Pac J Cancer Prev, 13, 6059-62.
  17. Maheshwari AV, Cheng EY (2010). Ewing sarcoma family of tumours. J Am Acad Orthop Surg, 18, 94-107.
  18. Oberlin O, Deley MC, Bui BN, et al (2001). Prognostic factors in localized Ewing's tumours and peripheral neuroectodermal tumours: the third study of the French Society of Paediatric Oncology (EW88 study). Br J Cancer, 85, 1646-54.
  19. Ogose A, Morita T, Hotta T, et al (1999). Brain metastases in musculoskeletal sarcomas. Jpn J Clin Oncol, 29, 245-7.
  20. Owens C, Laurence V, Benboubker L, et al (2013). Phase II study of cisplatin and oral VP16 in patients with refractory or relapsed Ewing sarcoma. Cancer Chemother Pharmacol, 71, 399-404.
  21. Raney RB, Asmar L, Newton WA, et al (1997). Ewing's sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol, 15, 574-82.
  22. Rud NP, Reiman HM, Pritchard DJ, et al (1989). Extraosseous Ewing's sarcoma: a study of 42 cases. Cancer, 64, 1548-53.<1548::AID-CNCR2820640733>3.0.CO;2-W
  23. Shannon Orr W, Denbo JW, Billups CA, et al (2012). Analysis of prognostic factors in extraosseous Ewing sarcoma family of tumours: review of St. Jude Children's Research Hospital experience. Ann Surg Oncol, 19, 3816-22.
  24. Sun XH, Hou WG, Zhao HX, et al (2013).Single Nucleotide Polymorphisms in the NER Pathway and Clinical Outcome of Patients with Bone Malignant Tumors. Asian Pac J Cancer Prev, 14, 2049-52.
  25. Tefft M, Vawter GF, Mitus A (1969). Paravertebral “round cell” tumours in children. Radiology, 92, 1501-9.
  26. Torchia EC, Jaishankar S, Baker SJ (2003). Ewing tumour fusion proteins block the differentiation of pluripotent marrow stromal cells. Cancer Res, 63, 3464-8.
  27. Tural D, Molinas Mandel N, Dervisoglu S, et al (2012). Extraskeletal Ewing's sarcoma family of tumours in adults: prognostic factors and clinical outcome. Jpn J Clin Oncol, 42, 420-6.
  28. Whelan JS, McTiernan A, Kakouri E, et al (2004). Carboplatinbased chemotherapy for refractory and recurrent Ewing's tumours. Pediatr Blood Cancer, 43, 237-42.
  29. Zagar TM, Triche TJ , Kinsella TJ (2008). Extraosseous Ewing's sarcoma: 25 years later. J Clin Oncol, 26, 4230-2.
  30. Zhang Q, Geng PL, Yin P, et al (2013). Down-regulation of long non-coding RNA TUG1 inhibits osteosarcoma cell proliferation and promotes apoptosis. Asian Pac J Cancer Prev, 14, 2311-5.

Cited by

  1. Survival of Patients with Ewing's Sarcoma in Yazd-Iran vol.15, pp.12, 2014,
  2. Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of the mediastinum: Significant response to chemoradiotherapy pp.1792-1082, 2014,
  3. Ewings Sarcoma-Primitive Neuroectodermal Tumour-Rare Extraosseous Presentation vol.05, pp.03, 2016,