Vol 14, No 6 (2018)
Review paper
Published online: 2019-03-15
Ewing sarcoma
Oncol Clin Pract 2018;14(6):392-398.
Abstract
Ewing sarcoma is a group of low-differentiated, high-grade, small-cell tumours. It is the third most common malignancy among primary bone tumours in adults. The prognosis of Ewing sarcoma is bad, and very often at the time of diagnosis the disease is highly advanced. For patients with localized disease, who have no metastases diagnosed at baseline, it is necessary to use combined treatment. It consists of induction chemotherapy (12–18 weeks) + local treatment (surgery ± radiotherapy or radiotherapy), and is followed by adjuvant consolidation chemotherapy. The whole treatment takes about 48–52 weeks. Advances in multidisciplinary treatment, including combined chemotherapy, surgery and radiotherapy have resulted in a significant improvement in patients’ quality of life and prognosis. Clinical trials are currently underway and new treatment standards are being developed.
Keywords: Ewing sarcomadiagnosistherapyprognosis
References
- Casali PG, Bielack S, Abecassis N, et al. ESMO Guidelines Committee, PaedCan and ERN EURACAN. Bone sarcomas: ESMO-PaedCan-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018; 29(Supplement_4): iv79–iv95.
- Gerrand C, Athanasou N, Brennan B, et al. British Sarcoma Group. UK guidelines for the management of bone sarcomas. Clin Sarcoma Res. 2016; 6: 7.
- Rutkowski P, Świtaj T. Bone sarcomas. Oncol Clin Pract. 2018; 14(3): 115–128.
- NCCN Clinical Practice Guidelines in Oncology: Bone Cancer. 2018; 1.
- Rutkowski P. Mięsaki kości. Viamedica Gdańsk. 2016.
- Le Deley MC, Paulussen M, Lewis I, et al. Cyclophosphamide compared with ifosfamide in consolidation treatment of standard-risk Ewing sarcoma: results of the randomized noninferiority Euro-EWING99-R1 trial. J Clin Oncol. 2014; 32(23): 2440–2448.
- Denbo JW, Shannon Orr W, Wu Y, et al. Timing of surgery and the role of adjuvant radiotherapy in ewing sarcoma of the chest wall: a single-institution experience. Ann Surg Oncol. 2012; 19(12): 3809–3815.
- Schuck A, Ahrens S, Paulussen M, et al. Local therapy in localized Ewing tumors: results of 1058 patients treated in the CESS 81, CESS 86, and EICESS 92 trials. Int J Radiat Oncol Biol Phys. 2003; 55(1): 168–177.
- Ladenstein R, Pötschger U, Le Deley MC, et al. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol. 2010; 28(20): 3284–3291.
- Schuck A, Hofmann J, Rübe C, et al. Radiotherapy in Ewing's sarcoma and PNET of the chest wall: results of the trials CESS 81, CESS 86 and EICESS 92. Int J Radiat Oncol Biol Phys. 1998; 42(5): 1001–1006.
- Bacci G, Palmerini E, Staals EL, et al. Ewing's sarcoma family tumors of the humerus: outcome of patients treated with radiotherapy, surgery or surgery and adjuvant radiotherapy. Radiother Oncol. 2009; 93(2): 383–387.
- Cotterill SJ, Ahrens S, Paulussen M, et al. Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. J Clin Oncol. 2000; 18(17): 3108–3114.
- Nesbit ME, Gehan EA, Burgert EO, et al. Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study. J Clin Oncol. 1990; 8(10): 1664–1674.
- Miser JS, Krailo MD, Tarbell NJ, 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(8): 694–701.
- Paulussen M, Craft AW, Lewis I, et al. European Intergroup Cooperative Ewing's Sarcoma Study-92. Results of the EICESS-92 Study: two randomized trials of Ewing's sarcoma treatment--cyclophosphamide compared with ifosfamide in standard-risk patients and assessment of benefit of etoposide added to standard treatment in high-risk patients. J Clin Oncol. 2008; 26(27): 4385–4393.
- Ladenstein R, Pötschger U, Le Deley MC, et al. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol. 2010; 28(20): 3284–3291.
- Whelan J, Le Deley MC, Dirksen U, et al. Euro-E.W.I.N.G.99 and EWING-2008 Investigators. High-Dose Chemotherapy and Blood Autologous Stem-Cell Rescue Compared With Standard Chemotherapy in Localized High-Risk Ewing Sarcoma: Results of Euro-E.W.I.N.G.99 and Ewing-2008. J Clin Oncol. 2018 [Epub ahead of print]: JCO2018782516.
- Gorlick R, Janeway KA, Adamson PC. Dose Intensification Improves the Outcome of Ewing Sarcoma. J Clin Oncol. 2018 [Epub ahead of print]: JCO2018793489.
- Euro Ewing 2012 protocol_version 5.0. http://www.euroewing.eu/clinical-trials/ee2012-trial/ee2012-update (2 Jun 2017).
- Zhou Z, Guan H, Duan X, et al. Zoledronic acid inhibits primary bone tumor growth in Ewing sarcoma. Cancer. 2005; 104(8): 1713–1720.
- Odri GA, Dumoucel S, Picarda G, et al. Zoledronic acid as a new adjuvant therapeutic strategy for Ewing's sarcoma patients. Cancer Res. 2010; 70(19): 7610–7619.
- Italiano A, Katz D. Cabozantinib in patients with advanced osteosarcomas and Ewing sarcomas: a French Sarcoma Group (FSG)/US National Cancer Institute phase II collaborative study. Presented at: the ESMO 2018 Congress; Munich, Germany: October 19-23, 2018. Abstract LBA67.