What the physician needs to know about Lynch syndrome

An update

Henry T. Lynch, Jane F. Lynch

Research output: Contribution to journalReview article

30 Citations (Scopus)

Abstract

The Lynch syndrome (hereditary nonpolyposis colorectal cancer [HNPCC]), is the most common form of hereditary colorectal cancer (CRC), accounting for 2% to 7% of all CRC cases. The next most common hereditary CRC syndrome is familial adenomatous polyposis (FAP), which accounts for less than 1% of all CRC. Lynch syndrome is of crucial clinical importance due to the fact that it predicts the lifetime risk for CRC and a litany of extra-CRC cancers (of the endometrium, ovary, stomach, small bowel, hepatobiliary tract, upper uroepithelial tract, and brain) through assessment of a well-orchestrated family history. A Lynch syndrome diagnosis is almost certain when a mutation in a mismatch repair gene - most commonly MSH2, MLH1, or, to a lesser degree, MSH6 - is identified. Once diagnosed, the potential for significant reduction in cancer-related morbidity and mortality through highly targeted surveillance may be profound. Particularly important is colonoscopy initiated at an early age (ie, 25 years) and repeated annually due to accelerated carcinogenesis. In women, endometrial aspiration biopsy and transvaginal ultrasound are important given the extraordinarily high risk for endometrial and ovarian carcinoma. These cancer control strategies have a major impact on at-risk family members once they have been counseled and educated thoroughly about Lynch syndrome's natural history and their own hereditary cancer risk.

Original languageEnglish
Pages (from-to)455-463
Number of pages9
JournalOncology
Volume19
Issue number4
StatePublished - 2005

Fingerprint

Hereditary Nonpolyposis Colorectal Neoplasms
Colorectal Neoplasms
Physicians
Endometrial Neoplasms
Hereditary Neoplastic Syndromes
Neoplasms
Adenomatous Polyposis Coli
DNA Mismatch Repair
Needle Biopsy
Colonoscopy
Natural History
Ovarian Neoplasms
Stomach
Carcinogenesis
Morbidity
Mutation
Mortality
Brain
Genes

All Science Journal Classification (ASJC) codes

  • Oncology

Cite this

What the physician needs to know about Lynch syndrome : An update. / Lynch, Henry T.; Lynch, Jane F.

In: Oncology, Vol. 19, No. 4, 2005, p. 455-463.

Research output: Contribution to journalReview article

Lynch, Henry T. ; Lynch, Jane F. / What the physician needs to know about Lynch syndrome : An update. In: Oncology. 2005 ; Vol. 19, No. 4. pp. 455-463.
@article{11a44d7c839b41fbbf4bddb0a27d53f6,
title = "What the physician needs to know about Lynch syndrome: An update",
abstract = "The Lynch syndrome (hereditary nonpolyposis colorectal cancer [HNPCC]), is the most common form of hereditary colorectal cancer (CRC), accounting for 2{\%} to 7{\%} of all CRC cases. The next most common hereditary CRC syndrome is familial adenomatous polyposis (FAP), which accounts for less than 1{\%} of all CRC. Lynch syndrome is of crucial clinical importance due to the fact that it predicts the lifetime risk for CRC and a litany of extra-CRC cancers (of the endometrium, ovary, stomach, small bowel, hepatobiliary tract, upper uroepithelial tract, and brain) through assessment of a well-orchestrated family history. A Lynch syndrome diagnosis is almost certain when a mutation in a mismatch repair gene - most commonly MSH2, MLH1, or, to a lesser degree, MSH6 - is identified. Once diagnosed, the potential for significant reduction in cancer-related morbidity and mortality through highly targeted surveillance may be profound. Particularly important is colonoscopy initiated at an early age (ie, 25 years) and repeated annually due to accelerated carcinogenesis. In women, endometrial aspiration biopsy and transvaginal ultrasound are important given the extraordinarily high risk for endometrial and ovarian carcinoma. These cancer control strategies have a major impact on at-risk family members once they have been counseled and educated thoroughly about Lynch syndrome's natural history and their own hereditary cancer risk.",
author = "Lynch, {Henry T.} and Lynch, {Jane F.}",
year = "2005",
language = "English",
volume = "19",
pages = "455--463",
journal = "Oncology",
issn = "0890-9091",
publisher = "UBM Medica Healthcare Publications",
number = "4",

}

TY - JOUR

T1 - What the physician needs to know about Lynch syndrome

T2 - An update

AU - Lynch, Henry T.

AU - Lynch, Jane F.

PY - 2005

Y1 - 2005

N2 - The Lynch syndrome (hereditary nonpolyposis colorectal cancer [HNPCC]), is the most common form of hereditary colorectal cancer (CRC), accounting for 2% to 7% of all CRC cases. The next most common hereditary CRC syndrome is familial adenomatous polyposis (FAP), which accounts for less than 1% of all CRC. Lynch syndrome is of crucial clinical importance due to the fact that it predicts the lifetime risk for CRC and a litany of extra-CRC cancers (of the endometrium, ovary, stomach, small bowel, hepatobiliary tract, upper uroepithelial tract, and brain) through assessment of a well-orchestrated family history. A Lynch syndrome diagnosis is almost certain when a mutation in a mismatch repair gene - most commonly MSH2, MLH1, or, to a lesser degree, MSH6 - is identified. Once diagnosed, the potential for significant reduction in cancer-related morbidity and mortality through highly targeted surveillance may be profound. Particularly important is colonoscopy initiated at an early age (ie, 25 years) and repeated annually due to accelerated carcinogenesis. In women, endometrial aspiration biopsy and transvaginal ultrasound are important given the extraordinarily high risk for endometrial and ovarian carcinoma. These cancer control strategies have a major impact on at-risk family members once they have been counseled and educated thoroughly about Lynch syndrome's natural history and their own hereditary cancer risk.

AB - The Lynch syndrome (hereditary nonpolyposis colorectal cancer [HNPCC]), is the most common form of hereditary colorectal cancer (CRC), accounting for 2% to 7% of all CRC cases. The next most common hereditary CRC syndrome is familial adenomatous polyposis (FAP), which accounts for less than 1% of all CRC. Lynch syndrome is of crucial clinical importance due to the fact that it predicts the lifetime risk for CRC and a litany of extra-CRC cancers (of the endometrium, ovary, stomach, small bowel, hepatobiliary tract, upper uroepithelial tract, and brain) through assessment of a well-orchestrated family history. A Lynch syndrome diagnosis is almost certain when a mutation in a mismatch repair gene - most commonly MSH2, MLH1, or, to a lesser degree, MSH6 - is identified. Once diagnosed, the potential for significant reduction in cancer-related morbidity and mortality through highly targeted surveillance may be profound. Particularly important is colonoscopy initiated at an early age (ie, 25 years) and repeated annually due to accelerated carcinogenesis. In women, endometrial aspiration biopsy and transvaginal ultrasound are important given the extraordinarily high risk for endometrial and ovarian carcinoma. These cancer control strategies have a major impact on at-risk family members once they have been counseled and educated thoroughly about Lynch syndrome's natural history and their own hereditary cancer risk.

UR - http://www.scopus.com/inward/record.url?scp=23844527874&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=23844527874&partnerID=8YFLogxK

M3 - Review article

VL - 19

SP - 455

EP - 463

JO - Oncology

JF - Oncology

SN - 0890-9091

IS - 4

ER -