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

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.

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  • Authors+Show Affiliations

    ,

    Department of Preventive Medicine, Creighton University School of Medicine, Omaha, Nebraska 68178.

    , , , , , , ,

    Source

    Diseases of the colon and rectum 31:5 1988 May pg 372-7

    MeSH

    Adult
    Aged
    Aged, 80 and over
    Colorectal Neoplasms, Hereditary Nonpolyposis
    Diagnosis, Differential
    Female
    Humans
    Male
    Middle Aged
    Pedigree

    Pub Type(s)

    Journal Article
    Research Support, U.S. Gov't, P.H.S.

    Language

    eng

    PubMed ID

    3366037

    Citation

    Lynch, H T., et al. "Differential Diagnosis of Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome I and Lynch Syndrome II)." Diseases of the Colon and Rectum, vol. 31, no. 5, 1988, pp. 372-7.
    Lynch HT, Watson P, Kriegler M, et al. Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). Dis Colon Rectum. 1988;31(5):372-7.
    Lynch, H. T., Watson, P., Kriegler, M., Lynch, J. F., Lanspa, S. J., Marcus, J., ... Cristofaro, G. (1988). Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). Diseases of the Colon and Rectum, 31(5), pp. 372-7.
    Lynch HT, et al. Differential Diagnosis of Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome I and Lynch Syndrome II). Dis Colon Rectum. 1988;31(5):372-7. PubMed PMID: 3366037.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II). AU - Lynch,H T, AU - Watson,P, AU - Kriegler,M, AU - Lynch,J F, AU - Lanspa,S J, AU - Marcus,J, AU - Smyrk,T, AU - Fitzgibbons,R J,Jr AU - Cristofaro,G, PY - 1988/5/1/pubmed PY - 2001/3/28/medline PY - 1988/5/1/entrez SP - 372 EP - 7 JF - Diseases of the colon and rectum JO - Dis. Colon Rectum VL - 31 IS - 5 N2 - 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. SN - 0012-3706 UR - https://www.unboundmedicine.com/medline/citation/3366037/Differential_diagnosis_of_hereditary_nonpolyposis_colorectal_cancer__Lynch_syndrome_I_and_Lynch_syndrome_II__ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=3366037.ui DB - PRIME DP - Unbound Medicine ER -