Description

Several families are affected by a diffuse gastric carcinoma occurring in close relatives. Some of these families will show evidence of a new or inherited mutation in CDH1 (epithelial cadherin, E-cadherin gene). A patient with a documented family history of diffuse gastric cancer may opt for prophylactic gastrectomy.


 

Inheritance: autosomal dominant

 

Parameters:

(1) number of close family members with gastric cancer

(2) youngest age of onset

(3) histologic features

Number of Affected Relatives in Family

Youngest Age

Histologic Features of Gastric Cancer

>= 1

< 35 years

diffuse

>= 2

< 50 years

tumor in person < 50 years of age diffuse

>= 3

NA

at least 1 diffuse

>= 2

NA

diffuse

from page 2361

 

where:

• Any patient being evaluated is included in the familial count.

 

Some kindreds have gastric cancer only while others will have cancers in other organs (breast, etc). In one group of families studied by Kaurah et al the risk of breast cancer (usually lobular type) in woman carriers was about 50%.

 


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