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The lymphatic system is a network of vessels, lymph nodes, and organs that helps the body fight infection and clear waste. Lymphoma is a type of cancer that starts in this system and involves your lymphocytes — a special type of white blood cell. In some cases, however, lymphoma starts in other tissues outside of the lymph nodes. These cases are called extranodal lymphomas.
Primary gastric lymphoma is an extranodal lymphoma that starts in the stomach. It’s different from secondary gastric lymphoma, which occurs when lymphoma develops in another location first and then spreads to the stomach.
In this article, we’ll discuss what you should know about gastric lymphoma, including the different types, potential symptoms, treatment options, and typical prognosis (outlook).
The stomach is the most common extranodal site, making up about 30 percent to 40 percent of extranodal lymphomas. Among all types of gastric (stomach) cancer, about 5 percent are lymphomas. Gastric lymphomas are more common in adults over age 50, but they can sometimes affect teens. According to StatPearls, men are two to three times more likely to be affected than women.
There are two main types of lymphomas: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Each type develops from different cells in the immune system. Many NHL cases begin in B-cell lymphocytes, but some originate in T-cell lymphocytes or natural killer (NK) cells.
Extranodal NHL often occurs in the stomach. Between 4 percent and 20 percent of non-Hodgkin lymphomas are gastric lymphomas.
Several subtypes of NHL may appear as gastric lymphomas. About 60 percent of gastric lymphoma cases are diffuse large B-cell lymphoma (DLBCL) — a high-grade (fast-growing) cancer. Around 40 percent of cases are mucosa-associated lymphoid tissue (MALT) lymphoma, also called extranodal “marginal zone B-cell lymphoma.” MALT lymphoma is a low-grade (slow-growing) disease.
Less often, gastric lymphoma can be mantle cell lymphoma, follicular lymphoma, or peripheral T-cell lymphoma. The type of gastric lymphoma can influence a person’s treatment options and prognosis (outlook). Gastric forms of HL are very rare, with only a few cases reported.
Some people with gastric lymphoma have no symptoms at first. Others may have vague symptoms or symptoms similar to those seen in more common stomach disorders.
Signs and symptoms of gastric lymphoma can include:
About 3 out of 10 people with gastric lymphoma also experience B symptoms — fever, drenching night sweats, and unexpected weight loss. People may also have lymphadenopathy (swollen lymph nodes, which may appear as hard lumps under the skin).
Different types of gastric lymphoma have been linked to various risk factors.
Up to 9 out of 10 people with MALT lymphoma have a Helicobacter pylori infection. This infection is often found in the stomach, and it doesn’t usually lead to cancer. In some cases, however, chronic (long-term) H. pylori infection can cause ongoing stomach inflammation, which increases the number of lymphocytes in this organ. Over time, these lymphocytes may turn cancerous. In rare cases, MALT lymphoma can turn into gastric DLBCL without proper treatment. DLBCL can also develop on its own.
Gastric lymphoma is also sometimes linked to other types of infections, including:
Gastric lymphomas have also been linked to inflammatory bowel diseases and celiac disease — a condition that develops when the body can’t properly process grains like wheat, rye, and barley.
Doctors use several tests to diagnose gastric lymphoma. These may include:
If lymphoma of the stomach is found, doctors will assess the lymphoma stage. Staging tells how far the cancer has spread in the gastrointestinal tract, lymph nodes, or other extranodal sites.
Basic blood tests can measure the levels of the three main types of blood cells: white blood cells, red blood cells, and platelets. Blood tests can also check levels of a protein called lactate dehydrogenase (LDH). People with lymphoma usually have increased levels of LDH. Very high levels often mean that the lymphoma is more aggressive.
Blood tests can also be used to check for infection by H. pylori or other bacteria or viruses. Infections may also be diagnosed with breath tests or stool tests.
To better understand what’s causing a stomach problem, doctors need to get a closer view. They usually do this with an upper endoscopy, also called an esophagogastroduodenoscopy. For this procedure, doctors use a thin tube with a camera on the end. They guide the tube through the throat to visualize tissues in the:
During an endoscopy, the doctor will usually take a tissue sample for a biopsy. The biopsy sample can be studied under a microscope to see whether cancer cells are present. If cancerous cells are found, further analysis can determine what type of cancer has developed.
After diagnosis, doctors typically use additional imaging tests to see how much cancer is in the stomach and whether it has spread. A CT scan is the most common test. Other imaging tests, like positron emission tomography (PET) scans (or, less commonly, MRI), may also be used in some cases.
Some doctors use the Lugano staging system for gastric lymphoma. This system doesn’t include a stage 3. The stages are:
Gastric lymphoma treatment varies based on the lymphoma type, stage, and grade and whether the cancer is related to an H. pylori infection. A person’s age, overall health, symptoms, and preferences also play a role in treatment decisions.
People with few or no symptoms may only require active monitoring — also known as a “watch and wait” approach — instead of immediate treatment.
Slow-growing, early-stage MALT lymphoma is highly treatable. Most people take antibiotics to clear an H. pylori infection. When the infection goes away, the lymphoma usually improves, too.
It may take a long time — a year or more — for the lymphoma to disappear after antibiotic treatment. Your doctor will monitor you closely during this time to keep an eye on the cancer. Several rounds of antibiotics and an additional endoscopy may be needed.
About 25 percent of gastric MALT lymphomas don’t go away after the H. pylori infection is treated. In addition, about 1 out of 10 people with this condition don’t have a bacterial infection in the first place. In these cases, other treatments may be necessary, including:
DLBCL is a high-grade lymphoma that requires more aggressive therapies. It’s often treated with a chemotherapy regimen called R-CHOP. These letters refer to:
When gastric DLBCL is detected in the early stages, doctors may recommend radiation therapy after going through chemo. People with advanced-stage gastric DLBCL usually only receive chemo.
Some newer immunotherapies called chimeric antigen receptor T-cell therapy may be used for advanced DLBCL, including gastric lymphoma. These include:
When gastric lymphoma is a form of HL, treatment usually starts with systemic (whole-body) chemotherapy. One common treatment, known as the ABVD regimen includes:
Another common regimen includes brentuximab with doxorubicin, vinblastine, and dacarbazine. Resection (surgery) isn’t routine, but doctors may recommend it in cases where bleeding, perforation (a hole in the digestive tract), or other complications occur. In general, most gastric lymphomas are treated without surgery, using antibiotics, chemoimmunotherapy (a combination of chemo and immunotherapy), or radiation instead.
A person’s prognosis depends on what type of gastric lymphoma they have. About 9 out of 10 people with MALT lymphoma live for 10 years or more after diagnosis.
Gastric DLBCL grows faster, but with modern treatment, outcomes have improved. Recently, as many as 73 percent of people are still alive five years after their diagnosis, and survival is even higher for early-stage disease.
Certain prognostic factors can lead to a poorer outlook, such as:
Overall, Hodgkin lymphoma is highly treatable, with long-term survival over 80 percent when using today’s treatments. Primary gastric Hodgkin lymphoma is very rare, and prognosis varies from person to person.
Your oncology team can give you the best picture of your outlook based on your specific situation.
On MyLymphomaTeam, people share their experiences with lymphoma, get advice, and find support from others who understand.
Are you or a loved one living with gastric lymphoma? Which symptoms have you experienced?
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Happy Easter to all my lymphoma friends!
I hope you all find joy today and hopefulness!
DonnaL
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