Familial adenomatous polyposis and extracolonic cancer

Henry T. Lynch, Alan G. Thorson, Rodney D. McComb, Barbara A. Franklin, Susan T. Tinley, Jane F. Lynch

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)2325-2332
Number of pages8
JournalDigestive Diseases and Sciences
Volume46
Issue number11
DOIs
StatePublished - 2001

Keywords

  • APC
  • Colorectal cancer
  • Desmoid
  • Familial adenomatous polyposis coli
  • Genes
  • Hepatoblastoma
  • Medulloblastoma
  • Thyroid cancer

ASJC Scopus subject areas

  • Physiology
  • Gastroenterology

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