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

Yoshihiro Nishida, Akira Kawai, Junya Toguchida, Akira Ogose, Keisuke Ae, Toshiyuki Kunisada, Yoshihiro Matsumoto, Tomoya Matsunobu, Kunihiko Takahashi, Kazuki Nishida, Toshihumi Ozaki

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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.

Original languageEnglish
JournalInternational Journal of Clinical Oncology
DOIs
Publication statusPublished - Jan 1 2019

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Keywords

  • Desmoid-type fibromatosis
  • Marginal resection
  • Registry
  • Treatment modality

ASJC Scopus subject areas

  • Surgery
  • Hematology
  • Oncology

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