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 Joseph Fitzgibbons, Giuseppe Cristofaro

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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
Pages (from-to)372-377
Number of pages6
JournalDiseases of the Colon and Rectum
Volume31
Issue number5
DOIs
StatePublished - May 1988

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Hereditary Nonpolyposis Colorectal Neoplasms
Differential Diagnosis
Colonic Neoplasms
Natural History
Endometrial Neoplasms
Age of Onset
Colorectal Neoplasms
Neoplasms
Colon
Physicians

All Science Journal Classification (ASJC) codes

  • Gastroenterology

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Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). / Lynch, Henry T.; Watson, Patrice; Kriegler, Mary; Lynch, Jane F.; Lanspa, Stephen J.; Marcus, Joseph; Smyrk, Tom; Fitzgibbons, Robert Joseph; Cristofaro, Giuseppe.

In: Diseases of the Colon and Rectum, Vol. 31, No. 5, 05.1988, p. 372-377.

Research output: Contribution to journalArticle

Lynch, Henry T. ; Watson, Patrice ; Kriegler, Mary ; Lynch, Jane F. ; Lanspa, Stephen J. ; Marcus, Joseph ; Smyrk, Tom ; Fitzgibbons, Robert Joseph ; Cristofaro, Giuseppe. / Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). In: Diseases of the Colon and Rectum. 1988 ; Vol. 31, No. 5. pp. 372-377.
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