Primary refractory Ewing sarcoma of the chest wall in an adult patient (case report). The potential and role of radiation therapy
https://doi.org/10.18027/2224-5057-2025-045
Abstract
Introduction: Ewing sarcoma is the second most common bone tumor, behind osteosarcoma, and is the most common bone tumor among children under 15 years of age. Quite rarely, these tumors occur in patients over 30 years of age and under 5 years of age. Ewing sarcoma of the chest wall is a rare malignant neoplasm, which is rarely reported in the scientific literature. An integrated approach combining chemotherapy, surgery, and radiation therapy is commonly used in the treatment of this neoplasm. However, there is no standard treatment for this tumor when the disease progresses during the first-line treatment.
Although modern combined approaches to treatment, including chemotherapy, surgery, and radiation therapy, help achieve disease control in some patients, the lack of standardized protocols for the treatment of refractory and recurrent forms results in significant difficulties in determining optimal therapeutic options. In real-world clinical practice, physicians have limited personal experience in managing such patients, which leads to variability in the chosen treatment plans for refractory disease and the need to make decisions based on limited data. The article discusses the main approaches to the treatment, as well as the potential and role of radiation therapy in the treatment of this disease and presents a case report of unresectable Ewing sarcoma of the chest wall in a 50-year-old male patient with local progression during first-line chemotherapy.
Supporting agencies: The article was prepared without sponsorship
About the Authors
А. А. Polyakov
Tomsk Regional Oncology Center
Russian Federation
Competing Interests:
Russian Federation
Aleksei Aleksandrovich Polyakov
634050; 115 Lenina Ave.; Tomsk
Competing Interests:
The authors declare that there are no possible conflicts of interest
P. А. Lushnikova
Tomsk Regional Oncology Center; Cancer Research Institute Tomsk “Tomsk national research medical center of the Russian Academy of Sciences»
Russian Federation
Competing Interests:
Russian Federation
Polina Aleksandrovna Lushnikova
634050; 115 Lenina Ave.; 634009; 5 Kooperativny Str.; Tomsk
Competing Interests:
The authors declare that there are no possible conflicts of interest
References
1. Ewing J., Diffuse endothelioma of bone. Reprinted from the Proceedings of the New York Pathological Society1921;21:17–24
2. Grier E. The ewing family of tumors: Ewing’s sarcoma and primitive neuroectodermal tumors. Pediatr Clin North Am 1997;44(4):991–1004. doi: 10.1016/s0031-3955(05)70541-1
3. Bellan D.G., Filho R.J., Garcia J.G., et al. Ewing’s sarcoma: epidemiology and prognosis for patients treated at the pediatric oncology institute. Rev Bras Ortop 2015;47(4):446–50. doi: 10.1016/S2255-4971(15)30126-9
4. Samburova N.V., Pimenov I.A., Zhevak T.N., Litvitsky P.F. Ewing’s sarcoma: molecular genetic pathogenic mechanisms. Current Pediatrics 2019;18(4):257–263 (In Russ.). doi: 10.15690/vsp.v18i4.2042
5. Chavan M., Dhakal S., Singh A., et al. Ewing sarcoma genomics and recent therapeutic advancements-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Pediatric Hematology Oncology Journal 2023;8:50–65. doi: 10.1016/j.phoj.2023.02.001
6. Sinichenkova K.Y., Roschin V.Y., Druy A.E. Molecular, biological and diagnostic features of Ewing sarcoma and undifferentiated small round cell sarcomas of bone and soft tissue. Bone and soft tissue sarcomas, tumors of the skin. 2021;13(1):24–31 (In Russ.). doi: 10.17650/2070-9781-2021-13-1-24-31
7. Semenova AI. Sarkoma Yuinga: kharakteristika zabolevaniya, osobennosti diagnostiki, lechebnaya taktika. Prakticheskaya onkologiya 2010;11(1):42–52 (In Russ.)
8. Tefft M., Vawter G.F., Mitus A. Paravertebral “round cell tumors in children”. Radiology 1969;92:1501–1509.
9. Abboud A., Masrouha K., Saliba M., et al. Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis. Oncol Lett 2021;21(5):354. doi: 10.3892/ol.2021.12615. PMID: 33747211
10. Carol C. Cheung, Rita A. Kandel, Robert S., et al. Extraskeletal Ewing Sarcoma in a 77-year-old woman. Arch Pathol Lab Med 2001;125(10):1358–1360. doi: 10.5858/2001-125-1358-EESIAY
11. Aliev M.D., Men T.H. Incidence of soft tissue sarcomas in Russia. Bone and soft tissue sarcomas, tumors of the skin 2013;3(3):3–9
12. Huh J., Kim K.W., Park S.J., et al. Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: A 17-year experience at a single institution. Korean J Radiol 2015;16(4):783–790. doi: 10.3348/kjr.2015.16.4.783
13. Applebaum M.A., Worch J., Matthay K.K., et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011;117(13):3027–32. doi: 10.1002/cncr.25840
14. Murphey M.D., Senchak L.T., Mambalam P.K., et al. From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics 2013;33(3):803–31. doi: 10.1148/rg.333135005
15. Ghandour M., Lehner B., Klotz M., et al. Extraosseous Ewing sarcoma in children : a systematic review and meta-analysis of clinicodemographic characteristics. Сhildren 2022;9(12):1859. d doi: 10.3390/children9121859
16. Fedeli M.A., Marras V., Fara A.M., et al. Primary Ewing sarcoma of the lung : A systematic review of the recent literature. Ann Diagn Pathol 2023;65:152152. doi: 10.1016/j.anndiagpath.2023.152152
17. Shek T.W., Chan G.C., Khong P.L., et al. Ewing sarcoma of the small intestine. J Pediatr Hematol Oncol 2001;23(8):530–532. doi: 10.1097/00043426-200111000-00013
18. Maeda M., Tsuda A., Yamanishi S., et al. Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child. Pediatr Blood Cancer 2008;50(1):180–3. doi: 10.1002/pbc.20831
19. Park J.Y., Lee S., Kang H.J., et al. Primary Ewing’s sarcoma primitive neuroectodermal tumor of the uterus : a case report and literature review. Gynecol Oncol 2007;106:427–432. doi: 10.1016/j.ygyno.2007.04.036
20. Petković M., Zamolo G., Muhvić D., et al. The first report of extraosseous Ewing’s sarcoma in the rectovaginal septum. Tumori 2002;88:345–346. doi: 10.1177/030089160208800419
21. Bosma S.E., Ayu O., Fiocco M., et al. Prognostic factors for survival in Ewing sarcoma : A systematic review, Surgical Oncol 2018;27(4):603–610. doi: 10.1016/j.suronc.2018.07.016
22. Van Mater D., Wagner L. Management of recurrent Ewing sarcoma: challenges and approaches. Onco Targets Ther 2019;27:12:2279–88 doi: 10.2147/OTT.S170585
23. Nesbit M.E. Jr, Gehan E.A., Burgert E.O. Jr, 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;8:1664–1674. doi: 10.1200/JCO.1990.8.10.1664
24. Grier H.E., Krailo M.D., Tarbell N.J., 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. doi: 10.1056/NEJMoa020890
25. PDQ Pediatric Treatment Editorial Board. Ewing Sarcoma and Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue Treatment (PDQ®): Health Professional Version. 2024 Sep 23. In: PDQ Cancer Information Summaries Internet. Bethesda (MD): National Cancer Institute (US); 2002
26. 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):168–77. doi: 10.1016/s0360-3016(02)03797-5
27. DuBois S.G., Krailo M.D., Gebhardt M.C., et al. Comparative evaluation of local control strategies in localized Ewing sarcoma of bone: a report from the Children’s Oncology Group. Cancer 2015;121(3):467–75. doi: 10.1002/cncr.29065
28. Choi Y., Lim D.H., Lee S.H., et al. Role of radiotherapy in the multimodal treatment of Ewing sarcoma family tumors. Cancer Res Treat 2015;47(4):904–12. doi: 10.4143/crt.2014.158
29. Zоllner S.K., Amatruda J.F., Bauer S., et al. Ewing sarcoma-diagnosis, treatment, clinical challenges and future perspectives. J Clin Med 2021;10(8):1685. doi: 10.3390/jcm10081685
30. Clinical Guidelines. Malignant neoplasms of bones and articular cartilage: osteosarcoma, Ewing’s sarcoma, 2024. Available at: https://cr.minzdrav.gov.ru/view-cr/70_2 (In Russ)
31. Lex J.R., Kurisunkal V., Kaneuchi Y., et al. Pelvic Ewing sarcoma: Should all patients receive pre-operative radiotherapy, or should it be delivered selectively? Eur J Surg Oncol 2021;47(10):2618–2626. doi: 10.1016/j.ejso.2021.05.027
32. Foulon S., Brennan B., Gaspar N., et al. Can postoperative radiotherapy be omitted in localised standard-risk Ewing sarcoma? An observational study of the Euro-E.W.I.N.G group. Eur J Cancer 2016;61:128–36. doi: 10.1016/j.ejca.2016.03.075
33. Indelicato D.J., Keole S.R., Shahlaee A.H., et al. Definitive radiotherapy for ewing tumors of extremities and pelvis: long-term disease control, limb function, and treatment toxicity. Int J Radiat Oncol Biol Phys 2008;72(3):871–7. doi: 10.1016/j.ijrobp.2008.02.023
34. La T.H., Meyers P.A., Wexler L.H., et al. Radiation therapy for Ewing’s sarcoma: results from Memorial Sloan-Kettering in the modern era. Int J Radiat Oncol Biol Phys 2006;64(2):544–50. doi: 10.1016/j.ijrobp.2005.07.299
35. Koontz B.F., Clough R.W., Halperin E.C. Palliative radiation therapy for metastatic Ewing sarcoma. Cancer 2006;106(8):1790–3. doi: 10.1002/cncr.21812
36. Leavey P.J., Laack N.N., Krailo M.D., et al. Phase III trial adding vincristine-topotecan-cyclophosphamide to the initial treatment of patients with nonmetastatic Ewing sarcoma: a Children’s Oncology Group Report. J Clin Oncol 2021;39(36):4029–4038. doi: 10.1200/JCO.21.00358
37. NCCN Clinical Practice Guidelines in Oncology. Bone Cancer. Version 1.2025. https://www.nccn.org/professionals/physician_gls/pdf/bone.pdf
38. Laskar S., Sinha S., Chatterjee A., et al. Radiation therapy dose escalation in unresectable Ewing sarcoma: final results of a phase 3 randomized controlled trial. Int J Radiat Oncol Biol Phys 2022;113(5):996–1002. doi: 10.1016/j.ijrobp.2022.04.024
39. Brown L.C., Lester R.A., Grams M.P., et al. Stereotactic body radiotherapy for metastatic and recurrent Ewing sarcoma and osteosarcoma. Sarcoma 2014;2014:418270. doi: 10.1155/2014/418270
40. DeLaney T.F., Trofimov A.V., Engelsman M., Suit H.D. Advanced-technology radiation therapy in the management of bone and soft tissue sarcomas. Cancer Control J 2005;12(1):27–35. doi: 10.1177/107327480501200104
41. Mounessi F.S., Lehrich P., Haverkamp U., et al. Pelvic Ewing sarcomas. Three-dimensional conformal vs. intensity-modulated radiotherapy. Strahlenther Onkol 2013;189(4):308–14. doi: 10.1007/s00066-012-0304-z
42. Su Z., Indelicato D.J., Mailhot R.B., Bradley J.A. Impact of different treatment techniques for pediatric Ewing sarcoma of the chest wall: IMRT, 3DCPT, and IMPT with/without beam aperture. J Appl Clin Med Phys 2020;21(6):100–107. doi: 10.1002/acm2.12870
43. Rombi B., DeLaney T.F., MacDonald S.M., et al. Proton radiotherapy for pediatric Ewing’s sarcoma: initial clinical outcomes. Int J Radiat Oncol Biol Phys 2012;82(3):1142–8. doi: 10.1016/j.ijrobp.2011.03.038.
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For citations:
Polyakov А.А., Lushnikova P.А. Primary refractory Ewing sarcoma of the chest wall in an adult patient (case report). The potential and role of radiation therapy. Malignant tumours. 2025;15(2):54-65. (In Russ.) https://doi.org/10.18027/2224-5057-2025-045
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