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The Desmoid Tumor Research Foundation

July 2019

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

Author Information

Nishida Y(1)(2), Kawai A(3), Toguchida J(4), Ogose A(5), Ae K(6), Kunisada T(7), Matsumoto Y(8), Matsunobu T(9), Takahashi K(10), Nishida K(10), Ozaki T(11).

(1)Dept of Orthopedic Surgery, Nagoya Univ Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. (2)Dept of Rehabilitation Medicine, Nagoya Univ Hospital, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. (3)Dept of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan. (4)Dept of Orthopaedic Surgery, Graduate School of Medicine, Kyoto Univ, 54 kawahara, Seigoin, Sakyo-ku, Kyoto, 606-8397, Japan. (5)Dept of Orthopedic Surgery, Uonuma Institute of Community Medicine, Niigata Univ Medical and Dental Hospital, 4132 Urasa, Minamiuonuma, Niigata, 949-7320, Japan. (6)Dept of Orthopedic Surgery, Cancer Institute Hospital, 3-8-31, Ariake, Koto-ku, Tokyo, 135-8550, Japan. (7)Dept of Medical Materials for Musculoskeletal Reconstruction, Okayama Univ Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikatacho, Kita-ku, Okayama, 700-8558, Japan. (8)Dept of Orthopedic Surgery, Kyushu Univ, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. (9)Dept of Orthopedic Surgery, Kyushu Rosai Hospital, 1-1Kokuraminami-ku, SonekitachoKitakyushu, Fukuoka, 800-0296, Japan. (10)Dept of Biostatistics, Nagoya Univ Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. (11)Dept of Orthopedic Surgery, Okayama Univ Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikatacho, Kita-ku, Okayama, 700-8558, Japan.

Publication

Int J Clin Oncol

Summary

In this study, tumor characteristics of desmoid-type fibromatosis (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.

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