Hereditary gastrointestinal cancer. Do you always remember them?

All surgeons in Portugal treat colorectal cancer (CRC) and gastric cancer (GC). Most of these are sporadic cases but some can represent an hereditary syndrome. The question is... While treating a gastrointestinal cancer do you study the possibility of being an hereditary syndrome? If not, you should!

In 2016, Hata et al. from the Department of Surgical Oncoloy of The University of Tokyo, Japan, published in the "Surgery Today" a review article entitled "Hereditary gastrointestinal cancer". In this paper the authors present a review of four major gastrointestinal syndromes:

1. Familial ademonatous poliposis (FAP)

2. Lynch syndrome (LS)

3. Hereditary diffuse gastric cancer (HDGC)

4. Li-Fraumeni syndrome (LFS)

In all these syndromes several cancers can occur, but in all four CRC and GC are a possibility. FAP has a penetrance of 100% of CRC until 60 years of age, decreasing to 30-70% in LS. Additionally, in both FAP and LS patients GC can occur. On the other hand, HDGC represents a hereditary GC syndrome which also causes CRC. Why is this important?

First, the treatment of sporadic and hereditary cancer can differ according to the hereditary syndrome diagnosed. Secondly, surveillance will not be equal to the general population. Third, there seems to be a potential link between CRC and GC regarding genetic pathways, even in sporadic cases.

Going to the third point, and according to the authors, Japanese individuals with family history of GC are reported to have a higher risk of CRC, and individuals with CRC have a higher risk of GC even if they do not have a known hereditary syndrome.

In the links below I am sharing a summary table for each of the hereditary gastrointestinal syndromes (does not dispense article reading):

• Table 1. Familial adenomatous poliposis

• Table 2. Lynch syndrome

• Table 3. Hereditary diffuse gastric cancer

• Table 4. Li-Fraumeni syndrome

I highly recommend reading this article since you must have a minimum knowledge of these syndromes if you are dealing with gastrointestinal cancer patients.

Link to the article:

Hata H, Yamamoto Y, Kiyomatsu T, Tanaka T, Kasama S, Nozawa H et al. Hereditary gastrointestinal cancer. Surg Today 2016; 46: 1115-1122.

Dr. Carlos Eduardo Costa Almeida

General Surgeon

#Surgery #Gastriccancer #Colorectalcancer #Hereditarycancer #Gastrointestinalcancer #FAP #Lynchsyndrome #HDGC #LiFraumeni