title: Familial adenomatous polyposis and extracolonic cancer.
abstract: Our purpose is to focus attention on the cancer family history, coupled with an understanding of the natural history and extracolonic tumor spectrum of familial adenomatous polyposis (FAP), through a family study. This family report provides an example of how colorectal cancer (CRC) can be prevented by knowledgeable gastroenterologists and colorectal surgeons who educate and compassionately counsel members of high-risk families so that their compliance with diagnostic screening and, ultimately, with protection through prophylactic colectomy, is achieved. A working pedigree of this extended family was constructed through interviews with the proband, followed by questionnaires sent to all primary and secondary relatives. Appropriately signed permission forms enabled us to secure pertinent medical and pathology records in order to ensure accuracy of historical information. Integral extracolonic tumors included medulloblastoma, papillary thyroid carcinoma, hepatoblastoma, and desmoid tumors. We conclude that, due in part to improved longevity as a result of being spared CRC, several family members have developed certain FAP integral extracolonic cancers.
authors.affiliation: Department of Preventive Medicine, Creighton University School of Medicine Omaha, Nebraska 68178, USA.
authors.forename: H T
authors.initials: HT
authors.lastname: Lynch
authors.forename: A G
authors.initials: AG
authors.lastname: Thorson
authors.forename: R D
authors.initials: RD
authors.lastname: McComb
authors.forename: B A
authors.initials: BA
authors.lastname: Franklin
authors.forename: S T
authors.initials: ST
authors.lastname: Tinley
authors.forename: J F
authors.initials: JF
authors.lastname: Lynch
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000235:genetics*; D002291:Carcinoma, Papillary / Q000235:genetics; D002528:Cerebellar Neoplasms / Q000235:genetics; D015179:Colorectal Neoplasms / Q000517:prevention & control*; D005260:Female; D018222:Desmoid Tumors / Q000235:genetics; D017491:Genes, APC; D018095:Germ-Line Mutation; D018197:Hepatoblastoma / Q000235:genetics; D006801:Humans; D008113:Liver Neoplasms / Q000235:genetics; D008297:Male; D008527:Medulloblastoma / Q000235:genetics; D010375:Pedigree; D013964:Thyroid Neoplasms / Q000235:genetics