Clinical features and treatment outcome of desmoid-type fibromatosis: based on a bone and soft tissue tumor registry in Japan
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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.
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.
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).
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.
KeywordsDesmoid-type fibromatosis Registry Treatment modality Marginal resection
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.
Compliance with ethical standards
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.
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 was waived because of the study based on the national registry data.
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