International Journal of Clinical Oncology

, Volume 9, Issue 4, pp 308–316

A clinical overview of familial adenomatous polyposis derived from the database of the Polyposis Registry of Japan

Authors

    • Department of Surgery, Kyoundo HospitalSasaki Institute
  • Kazuo Tamura
    • Department of Genetics, Institute for Advanced Medical ScienceHyogo College of Medicine
  • Takayuki Morita
    • Second Department of SurgeryHirosaki University Hospital
  • Takashi Hirai
    • Department of Digestive SurgeryAichi Prefecture Cancer Center
  • Hirotoshi Hasegawa
    • Department of SurgeryKeio University Hospital
  • Koichi Koizumi
    • Department of GastroenterologyTokyo Metropolitan Komagome Hospital
  • Kazuo Shirouzu
    • Department of SurgeryKurume Univerity Hospital
  • Kenichi Sugihara
    • Department of Digestive SurgeryTokyo Medical and Dental University Hospital
  • Takehira Yamamura
    • Second Department of SurgeryHyogo College of Medicine Hospital
  • Tetsuichiro Muto
    • Cancer Institute HospitalJapanese Foundation for Cancer Research
  • Joji Utsunomiya
    • Familial Cancer Center, Cancer Institute HospitalJapanese Foundation for Cancer Research Hospital
  • The Polyposis Committee, Japanese Society for Cancer of the Colon and Rectum
    • Familial Cancer Center, Cancer Institute HospitalJapanese Foundation for Cancer Research Hospital
REVIEW ARTICLE

DOI: 10.1007/s10147-004-0414-4

Cite this article as:
Iwama, T., Tamura, K., Morita, T. et al. Int J Clin Oncol (2004) 9: 308. doi:10.1007/s10147-004-0414-4

Abstract

The clinical situation of familial adenomatous polyposis (FAP) in Japan has changed in the period since the last analysis of data of the Japanese Polyposis Center. To reevaluate our data and elucidate the changes we analyzed the records of the 1390 FAP patients in 900 families registered with the Polyposis Committee of the Japanese Society for Cancer of the Colon and Rectum. In the 13-year period 1990–2003, clinical differences between men and women with FAP diminished. The postoperative prognosis was substantially better in patients without advanced colorectal cancer (stage ≧ T2) than in those with early cancer or no cancer. Mean age at death improved from 42.5 years in the period before 1990 to 46.0 years, and it was a result of a decreased proportion of deaths from colorectal cancer. The distribution of colorectal cancer in FAP patients was similar to that in the general population. Desmoid tumors accounted for about 10% of deaths in the recent 13 years (1990–2003). The cumulative risk of rectal cancer in the preserved rectum was 12% at 10 years and 23% at 15 years. The registry system in Japan revealed a new clinical situation in FAP patients, and the findings of this study will be useful to improve the prognosis of patients with FAP.

Key words

Familial adenomatous polyposisColorectal cancerSurvivalDesmoid tumorSurgical treatment of the colon and rectumRegistry

Copyright information

© The Japan Society of Clinical Oncology 2004