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

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

Research output: Contribution to journalArticle

136 Citations (Scopus)

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 (US)
Pages (from-to)143-160
Number of pages18
JournalCancer Genetics and Cytogenetics
Volume53
Issue number2
DOIs
StatePublished - 1991
Externally publishedYes

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Hereditary Nonpolyposis Colorectal Neoplasms
Natural History
Pathology
Lynch Syndrome II
Neoplasms
Colonic Neoplasms
Biomarkers
Endometrial Neoplasms
Colorectal Neoplasms
Differential Diagnosis
Research

ASJC Scopus subject areas

  • Cancer Research
  • Genetics
  • Molecular Biology

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Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, part I. / Lynch, Henry T.; Lanspa, Stephen; Smyrk, Thomas Christopher; Boman, Bruce; Watson, Patrice; Lynch, Jane.

In: Cancer Genetics and Cytogenetics, Vol. 53, No. 2, 1991, p. 143-160.

Research output: Contribution to journalArticle

Lynch, Henry T. ; Lanspa, Stephen ; Smyrk, Thomas Christopher ; Boman, Bruce ; Watson, Patrice ; Lynch, Jane. / Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, part I. In: Cancer Genetics and Cytogenetics. 1991 ; Vol. 53, No. 2. pp. 143-160.
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