Hereditary Diffuse Gastric Cancer (HDGC): Signs, Remedies, Prognosis
In 1997, the first case of Hereditary Diffuse Gastric Cancer (HDGC) was identified in a Māori family in New Zealand. Since then, this rare hereditary cancer syndrome has been found in various ethnic groups worldwide.
HDGC makes up less than 1% of all stomach cancers, with diffuse stomach cancer accounting for about 17%. This type of stomach cancer tends to cause a thickening of the stomach wall without forming a distinct tumor.
HDGC is an autosomal dominant genetic condition, meaning that receiving the gene from one parent is enough to develop the condition. The most common gene mutation associated with HDGC occurs in the CDH1 gene. Mutations in other genes such as STK11 and SMAD4 may also be associated with HDGC.
People with HDGC often don't show symptoms in the early stages. However, as the disease progresses, symptoms can include abdominal pain, nausea, vomiting, trouble swallowing, prolonged fullness after eating, loss of appetite, unintentional weight loss, and other symptoms. Similarly, women with an CDH1 mutation also have an estimated chance of developing lobular breast cancer by age 80, with symptoms such as thickening or hardening, swelling or fullness, change in texture or skin appearance, and changes in size or shape.
A biopsy can help differentiate HDGC from other stomach cancers. If symptoms of HDGC are present, doctors may order imaging tests like ultrasound, CT scan, or PET scan.
Genetic testing for the CDH1 gene should be considered in people who meet certain criteria, such as having a family history of stomach cancer and lobular breast cancer. People with family members with genes linked to HDGC can receive a genetic test to see if they carry the same gene.
The average age of onset for HDGC is around 40 years old, and most people are diagnosed before 50 years old. The estimated risk of developing gastric cancer by age 80 for people with an CDH1 mutation is significant.
For those with a confirmed HDGC diagnosis, prophylactic gastrectomy (stomach removal) can be an option to prevent the development of stomach cancer. However, this procedure can cause lifelong problems with digestion.
In the United States, the diagnosis frequency of HDGC is estimated at about 1 to 3% of all gastric cancer cases annually, but exact numbers per year are not well defined. Despite its rarity, understanding HDGC is crucial for early detection and prevention of this hereditary cancer syndrome.
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