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Phenotypic variation in colorectal adenoma/cancer expression in two families. Hereditary flat adenoma syndrome
Author(s) -
Lynch Henry T.,
Smyrk Thomas C.,
Lanspa Stephen J.,
Lynch Patrick M.,
Watson Patrice,
Strayhorn Patricia C.,
Bronson Earlene K.,
Lynch Jane F.,
Priluck Ira A.,
Appelman Henry D.
Publication year - 1990
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19900901)66:5<909::aid-cncr2820660516>3.0.co;2-h
Subject(s) - colorectal cancer , medicine , familial adenomatous polyposis , adenoma , cancer , phenotype , oncology , pathology , gastroenterology , genetics , biology , gene
Clinical, pathologic, and genetic studies on two colorectal cancer‐prone families have disclosed right‐sided colonic flat adenomas and colorectal cancer. Adenomatous polyp counts exceeded those found in hereditary nonpolyposis colorectal cancer (HNPCC) but were fewer than in familial adenomatous polyposis (FAP). Colon cancer occurred at a later age than in HNPCC or FAP and showed right‐sided predominance. The older age of patients with colonic cancer, the rightsided predominance of colon cancer, and the paucity of rectal adenomas make FAP unlikely. Vertical transmission of polyps and colon cancer fit the pattern of autosomal dominant inheritance. A characteristic feature of this phenotype is the predominance of flat adenomas. Molecular genetic studies, with careful description of phenotype, should help clarify classification.