Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications

Henry T. Lynch; Edibaldo Silva; Debrah Wirtzfeld; Pamela Hebbard; Jane Lynch; David G. Huntsman

doi:10.1016/j.suc.2008.04.006

Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications

Henry T. Lynch, Edibaldo Silva, Debrah Wirtzfeld, Pamela Hebbard, Jane Lynch, David G. Huntsman

Department of Preventive Medicine

Research output: Contribution to journal › Review article › peer-review

34 Scopus citations

Abstract

Hereditary diffuse gastric cancer (HDGC) is an autosomal dominantly inherited syndrome attributed to mutations of the E-cadherin gene, CDH1. There is no proven effective screening for early HDGC, and symptomatic disease is almost universally fatal. The only available effective option for CDH1 carriers is prophylactic total gastrectomy, but the variable age of onset of HDGC and the reduced penetrance (about 70%) of the CDHI gene further complicate patients' decision making.

Original language	English (US)
Pages (from-to)	759-778
Number of pages	20
Journal	Surgical Clinics of North America
Volume	88
Issue number	4
DOIs	https://doi.org/10.1016/j.suc.2008.04.006
State	Published - Aug 2008

All Science Journal Classification (ASJC) codes

Surgery

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10.1016/j.suc.2008.04.006

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title = "Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications",

abstract = "Hereditary diffuse gastric cancer (HDGC) is an autosomal dominantly inherited syndrome attributed to mutations of the E-cadherin gene, CDH1. There is no proven effective screening for early HDGC, and symptomatic disease is almost universally fatal. The only available effective option for CDH1 carriers is prophylactic total gastrectomy, but the variable age of onset of HDGC and the reduced penetrance (about 70%) of the CDHI gene further complicate patients' decision making.",

author = "Lynch, {Henry T.} and Edibaldo Silva and Debrah Wirtzfeld and Pamela Hebbard and Jane Lynch and Huntsman, {David G.}",

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T1 - Hereditary Diffuse Gastric Cancer

T2 - Prophylactic Surgical Oncology Implications

AU - Lynch, Henry T.

AU - Silva, Edibaldo

AU - Wirtzfeld, Debrah

AU - Hebbard, Pamela

AU - Lynch, Jane

AU - Huntsman, David G.

PY - 2008/8

Y1 - 2008/8

N2 - Hereditary diffuse gastric cancer (HDGC) is an autosomal dominantly inherited syndrome attributed to mutations of the E-cadherin gene, CDH1. There is no proven effective screening for early HDGC, and symptomatic disease is almost universally fatal. The only available effective option for CDH1 carriers is prophylactic total gastrectomy, but the variable age of onset of HDGC and the reduced penetrance (about 70%) of the CDHI gene further complicate patients' decision making.

AB - Hereditary diffuse gastric cancer (HDGC) is an autosomal dominantly inherited syndrome attributed to mutations of the E-cadherin gene, CDH1. There is no proven effective screening for early HDGC, and symptomatic disease is almost universally fatal. The only available effective option for CDH1 carriers is prophylactic total gastrectomy, but the variable age of onset of HDGC and the reduced penetrance (about 70%) of the CDHI gene further complicate patients' decision making.

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U2 - 10.1016/j.suc.2008.04.006

DO - 10.1016/j.suc.2008.04.006

M3 - Review article

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AN - SCOPUS:47849089452

SN - 0039-6109

VL - 88

SP - 759

EP - 778

JO - Surgical Clinics of North America

JF - Surgical Clinics of North America

IS - 4

ER -

Hereditary Diffuse Gastric Cancer: Prophylactic Surgical Oncology Implications

Abstract

All Science Journal Classification (ASJC) codes

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