Join us in Philadelphia this September for the DTRF "Together We Will" Weekend: - Register today!

The Desmoid Tumor Research Foundation

July 2020

Risk Factors of Local Recurrence After Surgery in Extra-Abdominal Desmoid-Type Fibromatosis: A Multicenter Study in Japan

Author Information

Yoshihiro Nishida (1,2), Shunsuke Hamada (3), Akira Kawai (4), Toshiyuki Kunisada (5), Akira Ogose (6), Yoshihiro Matsumoto (7), Keisuke Ae (8), Junya Toguchida (9), Toshifumi Ozaki (5), Akihiro Hirakawa (10), Toru Motoi (11), Tomohisa Sakai (2), Eisuke Kobayashi (4), Tabu Gokita (12), Takeshi Okamoto (9,13), Tomoya Matsunobu (14), Koki Shimizu (2,15), Hiroshi Koike (2).

(1)Dept of Rehabilitation Medicine, Nagoya Univ Hosp, Nagoya, Aichi, JP. (2)Dept of Orthopedic Surgery, Nagoya Univ Graduate School of Medicine, Nagoya, Aichi, JP. (3)Dept of Orthopedic Surgery, Aichi Cancer Center Hosp, Nagoya, JP. (4)Dept of Musculoskeletal Oncology, National Cancer Center Hosp, Tokyo, JP. (5)Dept of Orthopedic Surgery, Okayama Univ Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, JP. (6)Dept of Orthopedic Surgery, Uonuma Institute of Community Medicine, Niigata Univ Medical and Dental Hosp, Niigata, JP. (7)Dept of Orthopedic Surgery, Kyushu Univ, Fukuoka, JP. (8)Dept of Orthopedic Surgery, Cancer Institute Hosp, Tokyo, JP. (9)Dept of Orthopaedic Surgery, Kyoto Univ Graduate School of Medicine, Kyoto, JP. (10)Dept of Biostatistics and Bioinformatics, Graduate School of Medicine, The Univ of Tokyo, Tokyo, JP. (11)Dept of Pathology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hosp, Tokyo, JP. (12)Dept of Orthopedic Surgery, Saitama cancer center, Saitama, JP. (13)Dept of Orthopaedic Surgery, Otsu Red-cross Hosp, Shiga, JP. (14)Dept of Orthopedic Surgery, Kyushu Rosai Hosp, Fukuoka, JP. (15)Dept of Orthopaedic Surgery, Tono Kosei Hosp, Gifu, JP.

Publication

Cancer Sci

Summary

This study was undertaken to clarify the risk factors, including the mutation status of CTNNB1, for the local recurrence after surgery of the rare disease, desmoid-type fibromatosis. It was designed as a multi-institutional joint research project with seven major centers in Japan participating. Of 196 cases with specimens and medical records collected from the 7 institutions, 88 surgically treated ones were analyzed regarding clinicopathological prognostic factors including CTNNB1 mutation status. Excluding R2 cases (n=3), 5-year local recurrence free survival (LRFS) was 52.9%. No case had received pre- or post-operative radiotherapy. Univariate analysis revealed that extremity location (P<0.001) and larger size (?8cm, P=0.036) were significant adverse risk factors for LRFS. Multivariate analysis demonstrated that extremity location (P<0.001) was a significantly adverse factor in addition to recurrent tumor (P=0.041), S45F mutation (P=0.028), and R1 surgical margin (P=0.039). Pre-operative drug treatment including NSAIDs did not reduce the incidence of local recurrence (P=0.199). This is the first study to analyze the factors correlating with outcomes of surgical treatment including CTNNB1 mutation status in a relatively large number of cases from an Asian country. Tumor location was found to be the most influential prognostic factor for local recurrence, similar to the results from Western countries. The development of more sensitive method(s) for determination of CTNNB1 mutation is a priority for future study.

View Abstract