Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, part I

Henry T. Lynch, Stephen J. Lanspa, Thomas Smyrk, Bruce Boman, Patrice Watson, Jane Lynch

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Abstract

Hereditary nonpolyposis colorectal cancer (HNPCC) is common, accounting for about 4-6% of the total colorectal cancer burden. It is heterogeneous and appears to be delineated into two clinical subsets, Lynch syndromes I and II. Lynch syndrome I is characterized by an autosomal dominantly inherited proclivity to early onset colonic cancer with proximal predominance and an excess of multiple primary colonic cancer. Lynch syndrome II has all of these features plus extracolonic cancer sites, the most common of which is endometrial carcinoma. The lack of premonitory physical signs or biomarkers of HNPCC makes diagnosis difficult. A careful family history, tempered by an understanding of the clinical and pathologic features of HNPCC, is the key to its assessment. This paper reviews HNPCC's natural history, its integral extracolonic cancer associations, its differential diagnosis, surveillance, and management strategies. Attention is focused upon the need for biomarker research in the interest of improving control of HNPCC.

Original languageEnglish
Pages (from-to)143-160
Number of pages18
JournalCancer Genetics and Cytogenetics
Volume53
Issue number2
DOIs
Publication statusPublished - 1991

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All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Genetics
  • Molecular Biology

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