Hirotoshi Kobayashi1,2, Hideyuki Ishida3,4, Hideki Ueno3,5, Takao Hinoi3,6, Yasuhiro Inoue3,7, Fumio Ishida3,8, Yukihide Kanemitsu3,9, Tsuyoshi Konishi3,10, Tatsuro Yamaguchi3,11, Naohiro Tomita3,12, Nagahide Matsubara3,12, Toshiaki Watanabe3,13, Kenichi Sugihara3,14. 1. Department of Surgery, Tokyo Metropolitan Hiroo Hospital, 2-34-10 Ebisu, Shibuya-ku, Tokyo, 150-0013, Japan. h-kobayashi.srg2@tmd.ac.jp. 2. Study Group for Familial Adenomatous Polyposis (FAP) in the Japanese Society for Cancer of the Colon and Rectum, Tokyo, Japan. h-kobayashi.srg2@tmd.ac.jp. 3. Study Group for Familial Adenomatous Polyposis (FAP) in the Japanese Society for Cancer of the Colon and Rectum, Tokyo, Japan. 4. Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan. 5. Department of Surgery, National Defense Medical College, Saitama, Japan. 6. Department of Surgery and Institute for Clinical Research, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Hiroshima, Japan. 7. Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Mie, Japan. 8. Digestive Disease Center, Showa University, Northern Yokohama Hospital, Yokohama, Japan. 9. Colorectal Surgery Division, National Cancer Center Hospital, Tokyo, Japan. 10. Department of Gastroenterological Surgery, Gastroenterological Center, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan. 11. Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan. 12. Department of Surgery, Hyogo College of Medicine, Hyogo, Japan. 13. Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan. 14. Tokyo Medical and Dental University, Tokyo, Japan.
Abstract
PURPOSE: To investigate the incidence of colorectal cancer among familial adenomatous polyposis (FAP) patients by phenotype using the latest modalities. METHODS: We collected data on 303 patients who underwent surgery for FAP at one of 23 institutions between 2000 and 2012. The incidence of colorectal cancer was investigated by phenotype. RESULTS: Colorectal cancer was diagnosed in 115 (38.0 %) of the 303 patients. Overall, colorectal cancer with the attenuated, sparse, and profuse phenotypes was diagnosed at 30, 31, and 28 years of age, respectively, in 10 % of the patients and at 59, 48, and 41 years of age, respectively, in 50 % of the patients (P = 0.013). The patients with colorectal cancer were older than those without colorectal cancer for all phenotypes. The optimal cut-off age for predicting the development of colorectal cancer in the attenuated, sparse, and profuse phenotypes was 46, 31, and 27 years, respectively. CONCLUSIONS: Patients with profuse and sparse phenotypes should undergo prophylactic proctocolectomy before their mid-to-late 20 s. On the other hand, the timing and type of surgery for patients with attenuated FAP (AFAP) should be decided individually with reference to the colonoscopic findings.
PURPOSE: To investigate the incidence of colorectal cancer among familial adenomatous polyposis (FAP) patients by phenotype using the latest modalities. METHODS: We collected data on 303 patients who underwent surgery for FAP at one of 23 institutions between 2000 and 2012. The incidence of colorectal cancer was investigated by phenotype. RESULTS:Colorectal cancer was diagnosed in 115 (38.0 %) of the 303 patients. Overall, colorectal cancer with the attenuated, sparse, and profuse phenotypes was diagnosed at 30, 31, and 28 years of age, respectively, in 10 % of the patients and at 59, 48, and 41 years of age, respectively, in 50 % of the patients (P = 0.013). The patients with colorectal cancer were older than those without colorectal cancer for all phenotypes. The optimal cut-off age for predicting the development of colorectal cancer in the attenuated, sparse, and profuse phenotypes was 46, 31, and 27 years, respectively. CONCLUSIONS:Patients with profuse and sparse phenotypes should undergo prophylactic proctocolectomy before their mid-to-late 20 s. On the other hand, the timing and type of surgery for patients with attenuated FAP (AFAP) should be decided individually with reference to the colonoscopic findings.
Entities:
Keywords:
Cancer development; Familial adenomatous polyposis; Phenotype
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