title: The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  Our findings will provide clues that may help physicians in selecting the optimal strategy for this rare disease.
authors.affiliation: 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. yasinoue@clin.medic.mie-u.ac.jp.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Inoue
authors.affiliation: Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Ishida
authors.affiliation: Department of Surgery, National Defense Medical College, Saitama, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Ueno
authors.affiliation: Center for Minimally Invasive Surgery, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Hirotoshi
authors.initials: H
authors.lastname: Kobayashi
authors.affiliation: Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center, Komagome Hospital, Tokyo, Japan.
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Yamaguchi
authors.affiliation: Gastroenterological Center, Department of Gastroenterological Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan.
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Konishi
authors.affiliation: Department of Surgery, Hyogo College of Medicine, Hyogo, Japan.
authors.forename: Naohiro
authors.initials: N
authors.lastname: Tomita
authors.affiliation: Department of Surgery, Hyogo College of Medicine, Hyogo, Japan.
authors.forename: Nagahide
authors.initials: N
authors.lastname: Matsubara
authors.affiliation: Digestive Disease Center, Showa University Northern Yokohama Hospital, Kanagawa, Japan.
authors.forename: Fumio
authors.initials: F
authors.lastname: Ishida
authors.affiliation: Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan.
authors.forename: Takao
authors.initials: T
authors.lastname: Hinoi
authors.affiliation: Division of Colorectal Surgery, National Cancer Center Hospital, Tokyo, Japan.
authors.forename: Yukihide
authors.initials: Y
authors.lastname: Kanemitsu
authors.affiliation: Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Watanabe
authors.affiliation: Department of Surgical Oncology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sugihara
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000188:drug therapy / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003082:Colectomy; D015179:Colorectal Neoplasms / Q000209:etiology* / Q000517:prevention & control*; D003131:Combined Modality Therapy; D005260:Female; D018222:Desmoid Tumors / Q000209:etiology* / Q000517:prevention & control*; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D064887:Observational Studies as Topic; D016737:Proctocolectomy, Restorative; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal
keywords: Chemotherapy; Desmoid tumor; Familial adenomatous polyposis; Pharmacological treatment