Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II)

Henry T. Lynch, Patrice Watson, Mary Kriegler, Jane F. Lynch, Stephen J. Lanspa, Joseph Marcus, Tom Smyrk, Robert J. Fitzgibbons, Giuseppe Cristofaro

Research output: Contribution to journalArticle

88 Scopus citations

Abstract

Increasing recognition of the statistical burden posed by HNPCC (5 to 6 percent of all colorectal cancer) mandates that physicians have a better understanding of the genetics, natural history, and distinction between the hereditary site-specific variant (Lynch syndrome I) and the Cancer Family Syndrome (Lynch syndrome II). The authors report detailed cancer (all sites) family histories on two prototype families with Lynch syndrome I (Family R) and Lynch syndrome II (Family N), which have been under investigation for more than two decades. Emphasis is placed on shared clinicogenetic features; namely, early age of onset of colonic cancer (approximately age 44), multiple primary colonic cancer (24 percent of cases showed metachronous colonic cancer), predominance of proximal colonic cancer location (approximately 65 percent in the proximal colon), and vertical transmission consonant with an autosomal dominantly inherited factor. An increased predilection for extracolonic cancer, particularly endometrial carcinoma, occurs in Lynch syndrome II and is the primary basis for distinction from Lynch syndrome I. Surveillance and management programs must be wholly responsive to these natural history features.

Original languageEnglish (US)
Pages (from-to)372-377
Number of pages6
JournalDiseases of the Colon & Rectum
Volume31
Issue number5
DOIs
StatePublished - May 1 1988

All Science Journal Classification (ASJC) codes

  • Gastroenterology

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    Lynch, H. T., Watson, P., Kriegler, M., Lynch, J. F., Lanspa, S. J., Marcus, J., Smyrk, T., Fitzgibbons, R. J., & Cristofaro, G. (1988). Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). Diseases of the Colon & Rectum, 31(5), 372-377. https://doi.org/10.1007/BF02564888