Abstract
Background
The data about treatment results of Ewing sarcoma in adult patients are limited. The aim of our study was to analyze prognostic factors and outcomes of therapy in this group of patients.
Methods
Between 2000 and 2018, 180 patients at the age of > 18 years old diagnosed with Ewing sarcoma were treated in referral center according to multimodal protocols. In 50 patients (28%) treatment was initiated outside our hospital, and 23 of them had started recommended therapy after 3 months since the date of biopsy/unscheduled operation. We analyzed clinical prognostic factors and overall survival (OS).
Results
The median age was 28 years (18–67 years), primary tumor was localized axially in 114 patients (63%), metastases at presentation were detected in 51 pts (28%). 5-year OS rate was 65% for patients with localized disease, in metastatic disease it was 15%; the presence and the number of metastases was a prognostic factor. 5-year PFS was significantly better in patients treated at referral center (or when the patients were admitted to referral center within 3 months from the date of biopsy, which was performed outside referral center), comparing to patients treated initially outside referral center; 5-year PFS rates in total population were 28 and 13%, respectively. In terms of OS, unfavorable prognostic factor showing a statistical trend (p = 0.098) was lower dose density of neoadjuvant chemotherapy due to toxicity.
Conclusions
Approximately two-third of adult patients with localized Ewing sarcoma survive 5 years. In order to improve survival of this patients the multidisciplinary treatment in referral center is mandatory.
Similar content being viewed by others
References
Bellan DG, Filho RJ, Garcia JG et al (2012) Ewing’s sarcoma: epidemiology and prognosis for patients treated at the pediatric oncology. Rev Bras Ortop 47(4):446–450
Bernstein M, Kovar H, Paulussen M et al (2006) Pediatric oncology Ewing’s Sarcoma family of tumors: current management. Oncologist 11(5):503–519
Casali PG, Abecassis N, Bauer S et al (2018) Bone sarcomas: ESMO—PaedCan—EURACAN clinical practice guidelines for diagnosis, treatment and follow up. Ann Oncol 29:79–95
Krakorova DA, Kubackova K, Dusek L et al (2017) Advantages in prognosis of adult patients with ewing sarcoma: 11-years experiences and current treatment management. Pathol Oncol Res 24(3):623–630
Świtaj T, Jagodzińska-Mucha P (2018) Ewing's tumor. Oncol Clin Pract. https://doi.org/10.5603/OCP.2018.0052
Gerrand C, Athanasou N, Brennan B et al (2016) UK guidelines for the management of bone sarcomas. Clin Sarcoma Res. https://doi.org/10.1186/s13569-016-0047-1
Kilpatrick SE, Reith JD, Rubin B (2018) Ewing sarcoma and the history of similar and possibly related small round cell tumors: from whence have we come and where are we going? Adv Anatomic Pathol 25(5):314–326
Davis L, Malempati S (2014) Ewing sarcoma in adolescents and young adults: diagnosis and treatment. Clin Oncol Adolesc Young Adults 4:21–31
Esiashvili N, Goodman M, Marcus RB Jr (2008) Changes in incidence and survival of ewing sarcoma patients over the past 3 decades surveillance epidemiology and end results data. J Pediatr Hematol Oncol 30(6):425–430
Rutkowski P, Świtaj T (2018) Bone sarcomas. OwPK 14(3):115–128
Ahmed SK, Robinson SI, Okuno SH et al (2013) Adult ewing sarcoma: survival and local control outcomes in 102 patients with localized disease. Sarcoma 2013:681425
Gaspar N, Hawkins DS, Dirksen U et al (2015) Ewing sarcoma: current management and future approaches through collaboration. J Clin Oncol. https://doi.org/10.1200/JCO.2014.59.5256
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 institution. Clin Oncol 22(5):374–381
Duchman KR, Gao Y, Miller BJ (2015) Prognostic factors for survival in patients with Ewing’ s sarcoma using the surveillance, epidemiology, and end results (SEER) program database. Cancer Epidemiol. https://doi.org/10.1016/j.canep.2014.12.012
Van Mater D, Wagner L (2019) Management of recurrent Ewing sarcoma: challenges and approaches. Onco Targets Ther 27(12):2279–2288
Marina BNM, Pappo AS, Parham DM et al (2016) Chemotherapy dose-intensification for pediatric patients with Ewing’ s family of tumors and desmoplastic small round-cell tumors: a feasibility study at St. Jude Children’ s Research Hospital. J Clin Oncol 17(1):180
Anderton J, Moroz V, Marec-Bérard P et al (2020) Randomised I, Trial C, Ewing ND, Family S. International Randomised Controlled Trial for the Treatment of Newly Diagnosed Ewing’ s Sarcoma Family of Tumours Euro Ewing 2012. Trials. https://doi.org/10.1186/s13063-019-4026-8
Ladenstein R, Pötschger U, Le Deley MC et al (2010) Primary disseminated multifocal ewing sarcoma: results of the Euro-EWING 99 Trial. J Clin Oncol 28(20):3284–3291
Noujaim J, Jones RL, Swansbury J et al (2017) The spectrum of EWSR1-rearranged neoplasms at a tertiary sarcoma centre; assessing 772 tumour specimens and the value of current ancillary molecular diagnostic modalities. Nat Publ Gr 116(5):669–678
Bode-Lesniewska B, Fritz C, Exner GU et al (2019) EWSR1-NFATC2 and FUS-NFATC2 gene fusion-associated mesenchymal tumors: clinicopathologic correlation and literature review. Sarcoma. https://doi.org/10.1155/2019/9386390
Krystel-Whittemore M, Taylor MS, Rivera M et al (2019) Novel and established EWSR1 gene fusions and associations identified by next-generation sequencing and fluorescence in situ hybridization. Hum Pathol 93:65–73
Cotterill BSJ, Ahrens S, Paulussen M et al (2017) 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 18(17):3108–3114
Biermann JS, Chow W, Reed DR et al (2017) NCCN guidelines insights: bone cancer, version 2.2017. J Natl Compr Canc Netw 15(2):155–167
Bosma SE, Ayu O, Fiocco M et al (2018) Prognostic factors for survival in Ewing sarcoma: a systematic review. Surg Oncol 27(4):603–610
Hanson B, Ubert A, Dyer B et al (2013) Adult soft tissue Ewing’s sarcoma/primitive neuroectodermal tumor. Am Surg 79(7):E249–E250
Rosen G, Caparros B, Nirenberg A et al (1981) Cancer 47:2204–2213
Raciborska A, Bilska K, Drabko K et al (2014) Validation of a multi-modal treatment protocol for ewing sarcoma—a report from the Polish Pediatric Oncology Group. Pediatr Blood Cancer 61(12):2170–2174
Rao DN, Nair CN, Gopal R et al (1986) Adjuvant chemotherapy in Ewing’ s sarcoma. J Surg Oncol 32(2):76–78
Bacci G, Forni C, Longhi A et al (2004) Long-term outcome for patients with non-metastatic Ewing’ s sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. Eur J Cancer 40:73–83
Ducimetière F, Lurkin A, Ranchère-Vince D et al (2010) Incidence rate, epidemiology of sarcoma and molecular biology. Preliminary results from EMS study in the Rhône-Alpes region. Bull Cancer 97:629–641
Acknowledgements
The completion of this article could not have been possible without the participation and assistance of so many people whose names may not all be enumerated. Their contributions are sincerely appreciated and gratefully acknowledged. However, I would like to express their deep appreciation and indebtedness. The success and final outcome of this assignment required a lot of guidance and assistance. Thank you very much for all your help, support and good cooperation. This project was financed as a research grant by the MSC National Research Institute of Oncology.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
No authors has any conflict of interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Jagodzińska-Mucha, P., Ługowska, I., Świtaj, T. et al. The clinical prognostic factors and treatment outcomes of adult patients with Ewing sarcoma. Int J Clin Oncol 25, 2006–2014 (2020). https://doi.org/10.1007/s10147-020-01741-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10147-020-01741-7