TY  - CHAP
M1  - Book, Section
TI  - Polyposis Syndromes
A1  - Doherty, Gerard M.
PY  - 2010
T2  - Quick Answers Surgery
AB  - •  Familial  adenomatous  polyposis  (FAP)  (adenomatous  polyposis  coli)  is  a  rare  but  important  disease  because  colorectal  cancer  develops  before  age  40  in  nearly  all  untreated  patients•  FAP:  Autosomal  dominant•  APC  gene  localized  to  chromosome  5q21•  Thousands  of  polyps  (occasionally  fewer)  of  varying  size  and  configuration  are  present  in  the  colon  and  rectum•  Extracolonic  manifestations  are  associated  with  FAP:  -Endocrine  adenoma-Osteoma-Epidermoid  cyst-Small  bowel  adenoma-Visceral  malignancy-Desmoid  tumor-Thyroid  carcinoma-Hepatoblastoma•  Gardner  syndrome:  Variant  of  FAP  with  polyposis,  desmoid  tumors,  osteomas  of  mandible  or  skull,  and  sebaceous  cysts•  Turcot  syndrome:  Variant  of  FAP  with  polyposis  and  a  medulloblastoma  or  glioma•  Congenital  hypertrophy  of  retinal  pigment  epithelium  (always  bilateral,  more  than  4  lesions  on  each  side)  predicts  FAP  with  97%  sensitivity-This  abnormality  is  present  as  early  as  3  months  of  age  in  affected  members•  Polyps  begin  to  appear  at  puberty•  Cancer  develops  in  these  patients  at  a  mean  age  of  35  years  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesssurgery.mhmedical.com/content.aspx?aid=58098165
ER  -