Yoshihiro Nishida1,2, Akira Kawai3, Junya Toguchida4, Akira Ogose5, Keisuke Ae6, Toshiyuki Kunisada7, Yoshihiro Matsumoto8, Tomoya Matsunobu9, Kunihiko Takahashi10, Kazuki Nishida10, Toshifumi Ozaki11. 1. Department of Orthopedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. ynishida@med.nagoya-u.ac.jp. 2. Department of Rehabilitation Medicine, Nagoya University Hospital, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. ynishida@med.nagoya-u.ac.jp. 3. Department of Musculoskeletal Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan. 4. Department of Orthopaedic Surgery, Graduate School of Medicine, Kyoto University, 54 kawahara, Seigoin, Sakyo-ku, Kyoto, 606-8397, Japan. 5. Department of Orthopedic Surgery, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, 4132 Urasa, Minamiuonuma, Niigata, 949-7320, Japan. 6. Department of Orthopedic Surgery, Cancer Institute Hospital, 3-8-31, Ariake, Koto-ku, Tokyo, 135-8550, Japan. 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. 8. Department of Orthopedic Surgery, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. 9. Department of Orthopedic Surgery, Kyushu Rosai Hospital, 1-1Kokuraminami-ku, SonekitachoKitakyushu, Fukuoka, 800-0296, Japan. 10. Department of Biostatistics, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan. 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.
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.
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.
Authors: Dina Lev; Dhanasekaran Kotilingam; Caimiao Wei; Matthew T Ballo; Gunar K Zagars; Peter W T Pisters; Alexander A Lazar; Shreyaskumar R Patel; Robert S Benjamin; Raphael E Pollock Journal: J Clin Oncol Date: 2007-05-01 Impact factor: 44.544
Authors: Stephen X Skapek; James R Anderson; D Ashley Hill; David Henry; Sheri L Spunt; William Meyer; Simon Kao; Fredric A Hoffer; Holcombe E Grier; Douglas S Hawkins; R Beverly Raney Journal: Pediatr Blood Cancer Date: 2012-12-31 Impact factor: 3.167