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Outcomes of extraskeletal vs. skeletal Ewing sarcoma patients treated with standard chemotherapy protocol

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Abstract

Purpose

To compare the outcomes of extraskeletal and skeletal Ewing sarcomas treated with standard chemotherapy protocol.

Methods

We retrospectively collected data on primary localized skeletal and extraskeletal ES patients. Demographics and disease characteristics were compared between the two groups. The influence of presentation (skeletal vs. extraskeletal) on overall survival (OS) and local recurrence-free survival (LRFS) was assessed and compared by the log-rank test.

Results

A total of 120 patients were included; 29 (24%) had extraskeletal and 91 (76%) had skeletal ES. All patients received vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (VDC-IE) chemotherapy, with a plan for local control at week 12. At a median follow-up of 38 months, there was no difference in OS between skeletal and extraskeletal ES; 5-year OS 70% and 67% respectively, p = 0.96. Patients with extraskeletal ES had inferior 5-year LRFS compared to skeletal ES; 74% vs. 83%; p = 0.042. Local recurrence occurred at a higher frequency in the extraskeletal group; 28% vs. 11%, p = 0.034, although more extraskeletal patients received adjuvant radiotherapy; 73% vs. 36%, p = 0.01. Among patients who underwent surgery (n = 76), there was no difference in R0 resection rate (skeletal: 89%, extraskeletal: 86%, p = 0.52, or good ( ≥ 90%) tumor necrosis; skeletal: 54%, extraskeletal: 38%, p = 0.31.

Conclusion

Patients with localized extraskeletal ES have comparable OS outcomes to patients with skeletal ES utilizing the standard VDC-IE chemotherapy. However, extraskeletal patients are at significantly higher risk for local recurrence.

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Authors and Affiliations

  1. Medical Oncology Department, King Hussein Cancer Center, Queen Rania Al Abdullah St 202, Amman, 11941, Jordan

    S. Salah & S. Yaser

  2. Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan

    F. Abuhijla, A. Almousa & R. Abu-Hijlih

  3. Department of Pediatric Oncology and Hematology, King Hussein Cancer Center, Amman, Jordan

    T. Ismail, I. Sultan & H. Halalsheh

  4. Department of Orthopedic Surgery, King Hussein Cancer Center, Amman, Jordan

    A. Shehadeh & S. Abdelal

  5. Department of Pathology, King Hussein Cancer Center, Amman, Jordan

    O. Jaber

Authors
  1. S. Salah
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  2. F. Abuhijla
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  3. T. Ismail
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  4. S. Yaser
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  5. I. Sultan
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  6. H. Halalsheh
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  7. A. Shehadeh
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  8. S. Abdelal
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  9. A. Almousa
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  10. O. Jaber
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  11. R. Abu-Hijlih
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Contributions

Guarantor of integrity of the entire study: SS. Study concepts and design: SS, SY. Literature research: SS, RA-H, HH. Clinical studies: SS, RA-H, HH, FA. Experimental studies/data analysis: SS, RA-H, SY, AS, SA, AA, OJ. Statistical analysis: SS, IS. Manuscript preparation: SS. Manuscript editing: all authors.

Corresponding author

Correspondence to S. Salah.

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The authors have no conflict of interest to declare.

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The study was carried out to appropriate ethical standards.

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Salah, S., Abuhijla, F., Ismail, T. et al. Outcomes of extraskeletal vs. skeletal Ewing sarcoma patients treated with standard chemotherapy protocol. Clin Transl Oncol 22, 878–883 (2020). https://doi.org/10.1007/s12094-019-02202-y

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  • DOI: https://doi.org/10.1007/s12094-019-02202-y

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