Hereditary diffuse gastric cancer
Hereditary diffuse gastric cancer (HDGC) is an inherited disorder that significantly increases the risk of developing a specific, invasive form of stomach cancer.
Overview
Hereditary diffuse gastric cancer (HDGC) is an inherited disorder that greatly increases the chance of developing diffuse gastric cancer. In this form, there is no solid tumor; instead, malignant cells multiply underneath the stomach lining, making it thick and rigid. This invasive nature makes it highly likely that cancer cells will spread (metastasize) to other tissues, such as the liver or nearby bones.
Symptoms
Symptoms typically occur late in the disease and include:
- Stomach pain, nausea, and vomiting
- Difficulty swallowing (dysphagia)
- Decreased appetite and weight loss
If the cancer metastasizes, it may lead to an enlarged liver, jaundice, fluid buildup in the abdominal cavity (ascites), firm lumps under the skin, or broken bones.
Risk Factors
Gastric cancer in HDGC usually occurs in a person's late thirties or early forties. Some individuals also have an increased risk of other cancers, including:
- Lobular breast cancer in women
- Prostate cancer
- Colorectal cancer
Causes
Approximately 20 to 40 percent of individuals with HDGC have a mutation in the CDH1 gene. This gene provides instructions for making the protein E-cadherin, which helps cells stick together and acts as a tumor suppressor. When both copies of the CDH1 gene are mutated in a cell, the loss of functional E-cadherin prevents the protein from acting as a tumor suppressor, contributing to the development of cancer.
Inheritance
HDGC is inherited in an autosomal dominant pattern, meaning one copy of the altered CDH1 gene in each cell is sufficient to increase the risk of developing cancer.
