Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan

The Japanese Musculoskeletal Oncology Group cooperative study

Authors

  • Hideto Obata MD, PhD,

    1. Department of Orthopedics, Osaka University Graduate School of Medicine, Osaka, Japan
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    • The following investigators and their institutions participated in the study: A. Kawai and Y. Beppu (National Cancer Center); S. Iwata, T. Ishii, and S. Tatezaki (Chiba Cancer Center); H. Obata, T. Ueda, and H. Yoshikawa (Osaka University); T. Ozaki (Okayama University); S. Abe (Teikyo University); K. Tanaka and Y. Iwamoto (Kyusyu University); E. Nishida and H. Tsuchiya (Kanazawa University); A. Matsumine and A. Uchida (Mie University); T. Nakayama and J. Toguchida (Kyoto University); K. Morii and H. Yabe (Keio University); N. Araki (Osaka Medical Center for Cancer and Cardiovascular Diseases); Y. Nishida (Nagoya University); K. Honoki (Nara Medical University); Y. Yoshida (Nihon University Itabashi Hospital); A. Ogose (Niigata University); I. Kudawara (Osaka National Hospital); I. Shibuya (Teikyo University Mizonoguchi Hospital); K. Tsukide and Y. Yazawa (Tochigi Cancer Center); H. Watanabe (Gunma University); S. Shimose (Hiroshima University); H. Futani (Hyogo College of Medicine); M. Yokouchi (Kagoshima University); T. Wada (Sapporo Medical University); K. Kumagai (Nagasaki University); Y. Aoki (Himeji Red Cross Hospital); I. Baba (Osaka Medical College); S. Takashima (Fukui Medical University); K. Kushida (Kanagawa Cancer Center); and K. Ihara (Yamaguchi University).

  • Takafumi Ueda MD, PhD,

    Corresponding author
    1. Department of Orthopedics, Osaka University Graduate School of Medicine, Osaka, Japan
    • Department of Orthopedics, Osaka National Hospital, Kinki-Block Comprehensive Cancer Center, 2-1-14 Hoenzaka, Chuo-ku, Osaka 540-0006, Japan
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    • Fax: (011) 81-6-6943-6467

  • Akira Kawai MD, PhD,

    1. Division of Orthopedic Surgery, National Cancer Center Hospital, Tokyo, Japan
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  • Takeshi Ishii MD, PhD,

    1. Division of Orthopedic Surgery, Chiba Cancer Center, Chiba, Japan
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  • Toshifumi Ozaki MD, PhD,

    1. Science of Functional Recovery and Reconstruction, Graduate School of Medicine and Dentistry, Okayama University, Okayama, Japan
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  • Satoshi Abe MD, PhD,

    1. Department of Orthopedic Surgery, Teikyo University School of Medicine, Tokyo, Japan
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  • Kazuhiro Tanaka MD, PhD,

    1. Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
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  • Hiroyuki Tsuchiya MD, PhD,

    1. Department of Orthopedic Surgery, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan
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  • Akihiko Matsumine MD, PhD,

    1. Department of Orthopedic Surgery, Mie University Graduate School of Medicine, Tsu, Japan
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  • Hiroo Yabe MD, PhD

    1. Department of Orthopedic Surgery, Keio University School of Medicine, Tokyo, Japan
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  • Presented in part at the 5th meeting of the Asia Pacific Musculoskeletal Tumor Society, Izmir, Turkey, April 23–25, 2004 and at the 12th annual meeting of the Connective Tissue Oncology Society, Venice, Italy, November 2–4, 2006.

Abstract

BACKGROUND.

Ewing sarcoma family of tumors (ESFT) of bone is extremely rare in Japan. The objectives of the current study were to assess the clinical outcome and prognostic factors of patients with ESFT of bone in Japan and to compare them between Euro-American and Japanese populations.

METHODS.

The authors conducted a retrospective analysis of 243 patients who were treated for ESFT of bone in Japan between 1981 and 2003. Local therapy was surgery in 35% of patients, surgery combined with radiotherapy in 40% of patients, radiotherapy alone in 22% of patients, and no local treatment in 3% of patients. All but 3 patients received various regimens of multidrug chemotherapy.

RESULTS.

The median patient age was 16 years. The primary disease sites were the trunk in 53% of patients and the extremities in 47% of patients. Forty-one patients had metastases at presentation. The median follow-up was 66 months. A univariate survival analysis demonstrated that patients who had metastases at presentation, primary site in the trunk, age ≥16 years, tumor size ≥10 cm, tumor that responded poorly to induction chemotherapy, and local treatment with radiotherapy alone had a significantly worse event-free survival (EFS). A multivariate analysis further verified that the former 3 factors were significant adverse prognostic factors. Of 201 patients with localized disease, 45 patients who received current chemotherapy regimens that included ifosfamide and etoposide had a significantly better 5-year EFS rate (67.6%) compared with other patients.

CONCLUSIONS.

The clinical outcome of patients with localized ESFT of bone in Japan has improved markedly with the use of current chemotherapy regimens that include ifosfamide and etoposide and has become comparable to the outcomes observed in other major series of Euro-American patients. The prognostic factors are also almost identical. Cancer 2007 © 2007 American Cancer Society.

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