Understanding and Managing Hereditary Diffuse Gastric Cancer
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Receiving a diagnosis of hereditary diffuse gastric cancer (HDGC) can be an overwhelming and unsettling experience. The diagnosis may bring up emotions such as anxiety and fear. However, it is essential to understand that there are ways to manage and live with this genetic condition. With the right information, guidance and support from loved ones, you will be able to manage this disease and take care of your overall well-being.
What is Hereditary Diffuse Gastric Cancer?
Hereditary diffuse gastric cancer (HDGC) is a rare genetic condition that is associated with an increased risk of developing stomach (or gastric) cancer. This inherited syndrome predisposes you to an invasive and aggressive form of gastric cancer called diffuse-type gastric adenocarcinoma, which is also known simply as diffuse gastric cancer.
How is HDGC managed?
If you’ve been diagnosed with HDGC, managing the condition will focus on reducing your risk of developing diffuse gastric cancer itself. This can be done through a prophylactic total gastrectomy or endoscopic surveillance.
Prophylactic total gastrectomy (PTG)
If you have a germline CDH1 mutation, a family history of gastric cancer and are between 20 and 30 years old, your doctors may recommend that you undergo a prophylactic (preventative) total gastrectomy (PTG). This procedure, which involves surgically removing the entire stomach, is the only proven effective way of preventing diffuse gastric cancer in people with confirmed HDGC.
> Learn more about what goes on during a total gastrectomy
As with any operation, you will experience some complications after a PTG. This includes eating difficulties and rapid weight loss. This procedure will also be challenging, not only physically but emotionally and mentally as well. Knowing how to cope with these complications can be very helpful in ensuring a smooth recovery period.
> Learn more about the postoperative complications of a gastrectomy
Endoscopic surveillance
How an endoscopy is conducted to examine the upper gastrointestinal tract, including the stomach. Source: Shutterstock
If you are not a suitable candidate for PTG or if you decide to postpone or decline the procedure, you will require an intensive regimen of endoscopic surveillance with multiple random biopsies. This involves undergoing regular endoscopies and having your stomach lining biopsied to identify any cancerous cells.
Guidelines by the International Gastric Linkage Consortium (IGCLC) recommend a minimum of 30 biopsies taken from various anatomical regions of the stomach. This includes the cardia, fundus, body, antrum, transitional body-antrum zone and pre-pyloric area. The decision of where to biopsy within each region is left to your doctors' or endoscopist's discretion.
Tissue samples from these regions can be taken using standard biopsy forceps, ideally those with a "spike". This will ensure that the lamina propria, where signet ring cells may be present, is sampled.
However, due to the diffuse nature of this type of cancer, it may be difficult to identify cancerous cells during an endoscopy. For this reason, endoscopic exams can be ineffective at detecting early-stage diffuse gastric cancers.
When it comes to managing HDGC, it’s crucial to maintain communication with your doctors. They can provide you with information on the most effective ways to manage the condition and what’s best for you. Prioritizing your health and following your doctor’s advice can help ensure that you are taking the necessary steps to make informed decisions and improve your quality of life in the long run.