title: A clinical overview of familial adenomatous polyposis derived from the database of the Polyposis Registry of Japan.
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 > or = 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.
authors.affiliation: Department of Surgery, Kyoundo Hospital, Sasaki Institute, 1-8 Kanda-Surugadai, Chiyoda-ku, 101-0062, Tokyo, Japan. iwama@po.kyoundo.jp
authors.forename: Takeo
authors.initials: T
authors.lastname: Iwama
authors.forename: Kazuo
authors.initials: K
authors.lastname: Tamura
authors.forename: Takayuki
authors.initials: T
authors.lastname: Morita
authors.forename: Takashi
authors.initials: T
authors.lastname: Hirai
authors.forename: Hirotoshi
authors.initials: H
authors.lastname: Hasegawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Koizumi
authors.forename: Kazuo
authors.initials: K
authors.lastname: Shirouzu
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sugihara
authors.forename: Takehira
authors.initials: T
authors.lastname: Yamamura
authors.forename: Tetsuichiro
authors.initials: T
authors.lastname: Muto
authors.forename: Joji
authors.initials: J
authors.lastname: Utsunomiya
mesh_terms: D000236:Adenoma / Q000453:epidemiology* / Q000235:genetics / Q000601:surgery; D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000453:epidemiology* / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D015179:Colorectal Neoplasms / Q000453:epidemiology* / Q000235:genetics / Q000601:surgery; D019992:Databases as Topic; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012042:Registries; D012737:Sex Factors