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Primary Gastric Lymphoma

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Charles R. Thomas, Jr., MD, Professor and Chair, and Join Y. Luh, MD, FACP, Clinical Assistant Professor, Department of Radiation Medicine, OHSU Knight Cancer Institute, for assistance in the preparation of this report.

Synonyms of Primary Gastric Lymphoma

  • non-Hodgkin gastric lymphoma
  • primary Hodgkin's lymphoma of the stomach
  • stomach lymphoma, non-Hodgkins type

Disorder Subdivisions

  • diffuse large B-cell lymphoma (DLBCL) of the stomach
  • mucosa-associated lymphoid tissue (MALT) gastric lymphoma

General Discussion

Primary gastric lymphoma is a general term for a type of cancer that originates within the stomach. Approximately 90 percent of cases of primary gastric lymphoma are either mucosa-associated lymphoid tissue (MALT) gastric lymphoma or diffuse large B-cell lymphoma (DLBCL) of the stomach. MALT gastric lymphoma is often associated with infection with the Helicobacter pylori bacterium. Within the medical literature, significant controversy exists regarding the exact definition, classification and staging of primary gastric lymphoma.

The term lymphoma refers to cancer that arises in the lymphatic system. Functioning as part of the immune system, the lymphatic system helps to protect the body against infection and disease. It consists of a network of tubes known as lymph vessels that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph collects in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes. As lymph moves through the lymphatic system, it is filtered by a network of small structures known as lymph nodes that help to remove microorganisms (e.g., viruses, bacteria, etc.) and other foreign bodies from the bloodstream.

Most types of lymphoma result from errors in the production of a type of white blood cell (lymphocyte) or transformation of a single lymphocyte into a malignant cell. Abnormal, uncontrolled growth and proliferation of malignant lymphocytes may lead to enlargement of a specific lymph node region or regions; involvement of other lymphatic tissues, such as the spleen and bone marrow; and spread to other bodily tissues and organs, can lead to life-threatening complications. The specific symptoms (fever, night sweats, itchiness, etc.) and physical findings (weight loss, enlarged spleen, lumps over the neck or axilla, etc.) may vary from case to case, depending upon the extent and region(s) of involvement and other factors.

Most cases of primary gastric lymphoma are B-cell subtypes of non-Hodgkin lymphoma (NHL). NHL may be broadly classified into lymphomas that arise from abnormal B-lymphocytes (B-cell lymphoma) and those derived from abnormal T-lymphocytes (T-cell lymphoma). Most cases of lymphoma arise in the lymph nodes. When lymphoma arises outside the lymph nodes, it is referred to as extranodal lymphoma. Primary gastric lymphoma is the most common form of extranodal NHL.

NHL may also be categorized based upon certain characteristics of the cancer cells as seen under a microscope and how quickly they may tend to grow and spread. For example, NHL may be characterized as "low-grade" (or indolent), meaning it tends to grow slowly and result in few associated symptoms, or "intermediate-" or "high-grade" (aggressive) lymphomas, which typically grow rapidly, requiring prompt treatment. MALT gastric lymphoma is generally an indolent lymphoma; DLBCL of the stomach is generally an aggressive lymphoma. In some cases, individuals may have both forms of cancer at the same time.

Primary Gastric Lymphoma Resources

Organizations:

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