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Clinical features and treatment outcome of desmoid-type fibromatosis: based on a bone and soft tissue tumor registry in Japan

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Abstract

Background

Treatment modality of desmoid-type fibromatosis (DF) has changed from surgery with a wide surgical margin to conservative treatment. In this study, tumor characteristics of DF, transition of the treatment modality, and clinical outcome of surgical treatment were analyzed based on data obtained from the bone and soft tissue tumor registry established in Japan.

Methods

Data were collected as registration data and follow-up data. Five hundred and thirty registered cases of DF were identified, including 223 cases with follow-up data with or without surgical treatment.

Results

The number of registered patients increased gradually. The frequency of surgical treatment was gradually reduced year by year. The 3-year local recurrence free survival (LRFS) was 77.7%, with tumor location and size tending to correlate with LRFS. Interestingly, there was no significant difference in LRFS between wide and marginal margin (P = 0.34).

Conclusions

The treatment modality has shifted from surgical to conservative treatment, with risk factors for surgical treatment similar to those noted in previous studies. The National registry system is crucial for a rare disease such as DF, and in the future, a population based registry system should be established to better comprehend the actual status of DF.

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Acknowledgements

This work was supported in part by the Ministry of Health, Labor and Welfare of Japan, and the Ministry of Education, Culture, Sports, Science and Technology of Japan [Grant-in-Aid 17H01585 for Scientific Research (A)], and the National Cancer Center Research and Development Fund (29-A-3). We thank the hospitals and medical staff involved in the bone and soft tissue tumor registry. We also thank Ms Nakano and Ishihama for their support of the registry.

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Authors and Affiliations

  1. Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan

    Yoshihiro Nishida

  2. Department of Rehabilitation Medicine, Nagoya University Hospital, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan

    Yoshihiro Nishida

  3. Department of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan

    Akira Kawai

  4. Department of Orthopaedic Surgery, Graduate School of Medicine, Kyoto University, 54 kawahara, Seigoin, Sakyo-ku, Kyoto, 606-8397, Japan

    Junya Toguchida

  5. Department of Orthopedic Surgery, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, 4132 Urasa, Minamiuonuma, Niigata, 949-7320, Japan

    Akira Ogose

  6. Department of Orthopedic Surgery, Cancer Institute Hospital, 3-8-31, Ariake, Koto-ku, Tokyo, 135-8550, Japan

    Keisuke Ae

  7. Department of Medical Materials for Musculoskeletal Reconstruction, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikatacho, Kita-ku, Okayama, 700-8558, Japan

    Toshiyuki Kunisada

  8. Department of Orthopedic Surgery, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan

    Yoshihiro Matsumoto

  9. Department of Orthopedic Surgery, Kyushu Rosai Hospital, 1-1Kokuraminami-ku, SonekitachoKitakyushu, Fukuoka, 800-0296, Japan

    Tomoya Matsunobu

  10. Department of Biostatistics, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan

    Kunihiko Takahashi & Kazuki Nishida

  11. Department of Orthopedic Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikatacho, Kita-ku, Okayama, 700-8558, Japan

    Toshifumi Ozaki

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  1. Yoshihiro Nishida
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  2. Akira Kawai
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Corresponding author

Correspondence to Yoshihiro Nishida.

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Conflict of interest

Yoshihiro Nishida has no conflict of interest regarding this study, Akira Kawai has no conflict of interest regarding this study, Junya Toguchida has no conflict of interest regarding this study, Akira Ogose has no conflict of interest regarding this study, Keisuke Ae has no conflict of interest regarding this study, Toshiyuki Kunisada has no conflict of interest regarding this study, Yoshihiro Matsumoto has no conflict of interest regarding this study, Tomoya Matsunobu has no conflict of interest regarding this study, Kunihiko Takahashi has no conflict of interest regarding this study, Kazuki Nishida has no conflict of interest regarding this study, Toshifumi Ozaki has no conflict of interest regarding this study.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was waived because of the study based on the national registry data.

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Nishida, Y., Kawai, A., Toguchida, J. et al. Clinical features and treatment outcome of desmoid-type fibromatosis: based on a bone and soft tissue tumor registry in Japan. Int J Clin Oncol 24, 1498–1505 (2019). https://doi.org/10.1007/s10147-019-01512-z

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  • DOI: https://doi.org/10.1007/s10147-019-01512-z

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