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The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study

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Abstract

Purpose

Familial adenomatous polyposis (FAP)-associated desmoid tumor (DT) is sometimes life threatening. However, the optimal treatment for DTs has not been established. The aim of this study was to analyze the outcomes of surgical and pharmacological treatments for DT in Japanese FAP patients.

Methods

We retrospectively reviewed the data of 303 patients who underwent colectomy for FAP between 2000 and 2012. We analyzed 41 patients with DTs in which the location was apparent. The selection of treatment for intra-abdominal DTs was also evaluated according to Church’s classification.

Results

Surgery was frequently used to treat extra-abdominal DTs. Multimodal treatments, including surgery, and the administration of non-steroidal anti-inflammatory drugs, hormonal therapy, and chemotherapy were widely used for intra-abdominal DTs. The most effective pharmacological treatment was cytotoxic chemotherapy, which was associated with a response rate of 45.5% and a disease control rate of 72.7%. After a median follow-up period of 53.0 months, the 5-year DT-specific survival rate in patients with stage IV disease was 71.4%; in contrast, the rate in patients with other stages was 100%. Four-stage IV patients died of DT due to uncontrollable rapid progression. No cytotoxic chemotherapy was administered; however, incomplete resection was performed in three cases.

Conclusion

Our findings will provide clues that may help physicians in selecting the optimal strategy for this rare disease.

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Acknowledgements

The authors would like to acknowledge all of the patients and their families. In addition to the investigators in the author list, we acknowledge the following investigators who participated in this study: Koji Komori, Department of Gastroenterological Surgery Aichi Cancer Center Hospital, Aichi; Kenjiro Kotake, Department of Surgery, Tochigi Cancer Center, Tochigi; Takeshi Nagasaka, Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama; Hirotoshi Hasegawa, Department of Surgery, Keio University School of Medicine, Tokyo; Motoi Koyama, Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, Aomori; Yoshito Akagi, Department of Surgery, Kurume University School of Medicine, Kurume, Fukuoka; Toshimasa Yatsuoka, Department of Gastroenterological Surgery, Saitama Cancer Center, Saitama; Masataka Ikeda, Department of Surgery, National Hospital Organization, Osaka National Hospital, Osaka; Kensuke Kumamoto, Department of Organ Regulatory Surgery, Fukushima Medical University School of Medicine, Fukushima; Kiyotaka Kurachi, Department of Surgery 2, Hamamatsu University School of Medicine, Shizuoka;Toshiaki Watanabe, Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo; Kohji Tanakaya, Department of Surgery, Iwakuni Clinical Center, Yamaguchi; Kazuhiko Yoshimatsu, Department of Surgery, Tokyo Women’s Medical University Medical Center East, Tokyo.

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Authors and Affiliations

  1. Department of Gastrointestinal and Pediatric Surgery, Division of Reparative Medicine, Institute of Life Sciences, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie, 514-8507, Japan

    Yasuhiro Inoue

  2. Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan

    Hideyuki Ishida

  3. Department of Surgery, National Defense Medical College, Saitama, Japan

    Hideki Ueno

  4. Center for Minimally Invasive Surgery, Tokyo Medical and Dental University, Tokyo, Japan

    Hirotoshi Kobayashi

  5. Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan

    Tatsuro Yamaguchi

  6. Gastroenterological Center, Department of Gastroenterological Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan

    Tsuyoshi Konishi

  7. Department of Surgery, Hyogo College of Medicine, Hyogo, Japan

    Naohiro Tomita & Nagahide Matsubara

  8. Digestive Disease Center, Showa University Northern Yokohama Hospital, Kanagawa, Japan

    Fumio Ishida

  9. Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan

    Takao Hinoi

  10. Division of Colorectal Surgery, National Cancer Center Hospital, Tokyo, Japan

    Yukihide Kanemitsu

  11. Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

    Toshiaki Watanabe

  12. Department of Surgical Oncology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan

    Kenichi Sugihara

Authors
  1. Yasuhiro Inoue
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  2. Hideyuki Ishida
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  3. Hideki Ueno
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  4. Hirotoshi Kobayashi
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  6. Tsuyoshi Konishi
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  7. Naohiro Tomita
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  8. Nagahide Matsubara
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  9. Fumio Ishida
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  11. Yukihide Kanemitsu
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  12. Toshiaki Watanabe
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  13. Kenichi Sugihara
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Corresponding author

Correspondence to Yasuhiro Inoue.

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Funding

The present study was supported in part by a grant-in-aid for Cancer Research from the Ministry of Health, Labor and Welfare, and by the Japanese Society for Cancer of the Colon and Rectum.

Conflict of interest

The authors declare no conflicts of interest in association with the present study.

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Inoue, Y., Ishida, H., Ueno, H. et al. The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study. Surg Today 47, 1259–1267 (2017). https://doi.org/10.1007/s00595-017-1500-3

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  • DOI: https://doi.org/10.1007/s00595-017-1500-3

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