It is a cancer originating from b cells in the marginal zone of the malt, and is also called extranodal marginal zone b cell lymphoma. Several recent publications have furthered the understanding of gastric malt lymphoma pathogenesis, clinical behavior, and. However, the presence of a serum monoclonal component has not been associated with the disseminated disease. This extranodal lymphoma develops from blymphocytes of the margi. Marginal zone lymphomas are typically not very aggressive and have better prognosis compared to other bcell lymphomas. Nov 27, 2017 malt lymphoma affects the lymphatic system, which is responsible for the immunity, making it weak and prone to infections. A pproximately 40% of all nonhodgkins lymphomas occur in extranodal locations. Malt lymphoma represents about 7% of all nonhodgkin lymphomas and can arise at any extranodal site. Gastric malt lymphoma is a distinct entity related to helico bacter pylori gastritis. Mature bcell and plasma cell neoplasms lowgrade bcell lymphoma follicular lymphoma. It develops in lymphatic tissue in the mucous membranes or tissues that line body organs or cavities. There is now compelling evidence that gastric malt lymphoma is caused by infection with h pylori.
Because gastric malt lymphoma usually is caused by a bacteria called helicobacter pylori h. Effects of antimicrobial therapy on gastric malt lymphoma in mice. Many of our members have or had gastric malt and began treatment with the antibiotic therapy. Stomach biopsy showed a marginal zone bcell lymphoma maltoma of the greater curvature and hp.
A phase 2 study of rituximab plus lenalidomide for mucosaassociated lymphoid tissue lymphoma. A case of api2malt1positive gastric malt lymphoma with. Prognosis depends upon the histology, grade and stage see grading and staging, and treatment modality employed, but the overall fiveyear survival rate is approximately 60 percent. Therefore, malt lymphomas are often stage i and highly curable. Dissemination of malt type lymphoma to bone marrow and peripheral blood simultaneously with the presence of tlarge granular cell leukemia tlgl has rarely been. Primary gastric lymphoma lymphoma that originates in the stomach itself is an uncommon condition, accounting for less than 15% of gastric malignancies and about 2% of all lymphomas. Malt lymphomas represent the most frequent istotype in lowgrade malignant primary gastric lymphomas table i. Gy in 20 equal daily fractions to the bladder and tolerated. This bacterial infection not only triggers the acquisition of malt in the gastric mucosa, a prerequisite for lymphoma development, 21,22 but also plays a critical role in malignant transformation and subsequent clonal expansion of the transformed cells. Threequarters of gastric malt lymphomas regress after the eradication of h. Mucosa associated lymphoid tissue malt lymphoma, also called extranodal marginal zone bcell lymphoma, is a type of lowgrade extranodal lymphoma. The malt was found while doing stomach biopsies and there are no tumors at this time. Pathology outlines extranodal marginal zone lymphoma of.
Mucosa associated lymphoid tissue malt lymphoma, also called extranodal marginal zone bcell lymphoma, is a type of lowgrade extranodal lymphoma chronic infectioninflammation has been implicated in the pathogenesis, for example helicobacter pylori. The main treatment of malt lymphoma include radiotherapy, chemotherapy, surgery or. Malt is classified as an extranodal marginal zone lymphoma. Although extragastrointestinal involvement has been rarely reported, malt type lymphomas are most common in the stomach, followed by small intestine. Malt lymphoma is called extranodal because it starts in tissues or organs outside of the lymph nodes.
Primary gastric lymphoma nord national organization for. According to some epidemiologic studies, maltomas are slightly more common in females than in males and are more common in whites than in blacks. Disseminated gastric malt lymphoma with monoclonal gammopathy. The majority are of the diffuse large b cell type but extranodal marginal zone b cell lymphoma of mucosa associated lymphoid tissue malt lymphoma is the commonest extranodal small b cell nonhodgkins lymphoma. Mucosaassociated lymphoid tissue malt lymphoma patient. It is a slowgrowing bcell nonhodgkins lymphoma affecting adults. Malt lymphomas comprise approximately 5% of all nonhodgkins lymphomas in the united states.
Diagnostic accuracy of petct in patients with extranodal marginal zone malt lymphoma. Approximately 8% of all nonhodgkin lymphomas are extranodal marginal zone b cell lymphoma of mucosa associated lymphoid tissue malt, also known as malt lymphoma, which was first described in 1983 by isaacson and wright. Mature bcell and plasma cell neoplasms lowgrade bcell lymphoma follicular lymphoma published date. Gastric mucosaassociated lymphoid tissue lymphoma and helicobacter pylori infection. Gastric mucosaassociated lymphoid tissue malt lymphoma represents approximately 40% of gastric lymphomas, and its incidence is increasing. Gastric mucosaassociated tissue lymphoma malt lymphoma is a rare mature bcell neoplasm associated with h. Gastric mucosaassociated lymphoid tissue malt lymphoma is closely associated with helicobacter pylori hp infection find. Helicobacter pylori is the major cause of gastric malt lymphoma and is present in 7590% of cases. Gastric marginal zone lymphoma of mucosaassociated lymphoid tissue. I have nodal marginal zone lymphoma stage iv and malt lymphoma in my stomach. Second line therapy for malt lymphomas is usually chemotherapy with a single agent, and complete response rates of greater than 70% have been reported. In the literature the detection rate of 18ffdgpetct in patients with gastric malt lymphoma is variable, and the reason for this heterogeneity is not still clear. However, the stomach is a very common extranodal site for lymphomas lymphomas originate elsewhere and metastasise to the stomach. Radiation oncologynhlmalt wikibooks, open books for an.
Overview on the management of nongastric malt lymphomas. It is associated with malt lymphoma of the orbit, of the thyroid and skin, whereas it was not found in malt lymphoma of the stomach, of the salivary gland and in other forms of mzbcl. Extranodal marginal zone lymphoma, also called low grade bcell lymphoma of mucosaassociated lymphoid tissue malt, is an extranodal lymphoma that arises in a number of epithelial tissues. Extranodal marginal zone lymphoma of mucosaassociated lymphoid tissue malt is a bcell nonhodgkin lymphoma nhl. Gastric lymphoma originating from mucosaassociated lymphoid tissue malt is typically a. Gastric marginal zone lymphoma of mucosaassociated lymphoid tissue malt type, commonly known as gastric malt lymphoma, is derived from marginal zone bcells, accounts for 3040% of all extranodal lymphomas and represents 28% of all gastric cancers. Helicobacter pylori infection is a pre malt lymphoma condition. H pylori eradication leads to complete remission in 80% of low grade stage e1 lymphomas, with a yearly recurrence rate of. Other sites that may be involved include the salivary glands, eyes, lung, i. The most common chromosomal translocation in malt lymphoma is t11. Malt lymphoma is a lowgrade indolent type of bcell nonhodgkin lymphoma. The most common histological subtypes are diffuse large bcell and marginal zone bcell nhl of the mucosaassociated lymphoid tissue malt type. What your doc has planned with antibiotics, then endoscopy and scans, is a good plan for gastric malt. Malt lymphoma occurs in people from all geographic areas and all ethnic and racial.
Various microorganisms may have an etiologic link to non gastric malt lymphomas arising in other anatomical sites. The pathological diagnosis of gastric malt lymphoma can be reached by. Malt lymphomas arise at a wide range of different extranodal sites, with the highest frequency in the stomach, followed by lung, ocular adnexa, and thyroid, and with a low. The endoscopic findings of gastric malt lymphoma are variable, making its diagnosis very difficult. Antigendependent progression of mucosaassociated lymphoid tissue malt type lymphoma in the stomach. There are four main treatment options for orbital lymphoma. Sep 12, 2016 the role of 18ffdgpetct in evaluating gastric malt lymphoma is still controversial. Although the sex ratio incidence is essentially equal, gastric lymphoma appears to be slightly more prevalent in males male. According to worldwide literature, nhl accounts for 23% of all malignancies in the world and malt lymphomas comprise approximately 5% of all nhls. Antibiotics are used to treat gastric malt lymphoma. The presentation of a malt lymphoma in the bladder is exceedingly rare. Our aim was to investigate the particular metabolic behavior of these lymphoma. Jan 17, 2019 perry c, herishanu y, metzer u, et al. The onequarter that are unresponsive to antibiotics carry a characteristic chromosomal translocation or are at an advanced stage.
An early diagnosis for gastric malt lymphoma is important, but not easy due to nonspecific symptoms and endoscopic findings. In the past decade, combinedmodality therapy with chemotherapy followed by rt was introduced for largecell lymphoma, while rt alone was used for mucosaassociated lymphoid tissue malt lymphoma. Pathologically, see lymphoepithelial lesions as the malignant lymphoma cells invade and destroy normal epithelium such as glands. Pdf diagnosis and treatment of gastric malt lymphoma. A malt lymphoma can develop almost anywhere in the body, but it most commonly develops in the stomach. Mucosaassociated lymphoid tissue lymphomas maltomas. Jan 17, 2019 the median age of onset for mucosaassociated lymphoid tissue malt lymphomas maltomas is 65 years.
Malt lymphoma symptoms, diagnosis and treatment bmj best. Malt lymphoma, second malignancy, api2malt1, dlbcl this is an open access article distributed under the creative commons attributionnoncommercialnoderivatives 4. The coexistance of malt lymphoma and diffuse large bcell lymphoma should not be reported as high grade malt because extranodal marginal zone malt lymphoma is an indolent lymphoma. Primary gastric lymphoma is a general term for a type of cancer that originates within the stomach.
Diagnosis and management of orbital lymphoma american. Sep 16, 2018 gastric malt mucosaassociated lymphoid tissue lymphoma is a marginal zone lymphoma mzl of the stomach. Extranodal marginal zone lymphomas tend to home to a certain tissue and remain localized. Helicobacter pylori infection is a premalt lymphoma condition. Gastric malt lymphoma is a form of nonhodgkin lymphoma that arises from tissue that forms the lining of the stomach. The nonhodgkin lymphoma subtype of marginal zone lymphoma represents a group of lymphomas that have been historically classified together because they appear to arise from postgerminal center marginal zone b cells and share a similar immunophenotype.
How gastric malt lymphoma is treated verywell health. Based on these findings, the egils european gastrointestinal lymphoma study european consensus report of 2011 recommends eradication therapy of h. Indeed chronic antigenic stimulation causes genetic instability and clonal growth of malt. Primary gastric lymphoma pathogenesis and treatment. Trisomy 3 and trisomy 18 were reported in lowgrade as well as highgrade malt lymphoma. Malt lymphoma maltoma is a form of lymphoma involving the mucosaassociated lymphoid tissue malt, frequently of the stomach, but virtually any mucosal site can be afflicted. Primary gastric pg lymphomas are generally nonhodgkin lymphomas nhl. Clinicopathologic characteristics and treatment of marginal zone lymphoma of mucosaassociated lymphoid tissue malt lymphoma. Approximately 50% of malt lymphomas occur in the gi tract and about 85% of these occur in the stomach.
The main causes of malt lymphoma is chronic infections and persistent inflammation in the body. Treatment should be tailored to the affected organ and may consist of surgery, chemotherapy andor radiation therapy 2. Gastric malt lymphomas are clinically and histologically quite distinct from comparable lowgrade bcell lymphomas of lymph nodes. Indolent bcell lymphomas that are supposed to derive from the marginal zone marginal zone. Outcome and quality of life favour a conservative treatment of patients with primary gastric lymphoma. The term malt lymphoma was first suggested by isaacson and wright in 1983 to describe low grade gastric b cell lymphoma and immunoproliferative small intestinal disease. Various microorganisms may have an etiologic link to nongastric malt lymphomas arising in other anatomical sites. The stomach is the most frequently involved organ, and in most cases detected in 90%, there is a strong association between gastric malt lymphoma and chronic infection. Approximately 90 percent of patients of primary gastric lymphoma are either mucosaassociated lymphoid tissue malt gastric lymphoma or diffuse large bcell lymphoma dlbcl of the stomach. Disseminated gastric malt lymphoma with monoclonal. Gastric malt lymphoma american society of hematology. Gastric bcell mucosaassociated lymphoid tissue malt. Nh malt lymphoma of the stomach cancer survivors network. Mucosa associated lymphoid tissue lymphoma of the colon.
Right now they are waiting for a bacterial overgrowth in my sm intestine to clear and then they want to treat with chemo because the marginal zone nhl is on the. May 28, 2014 malt lymphoma is a subtype of nonhodgkins lymphoma with its own specific pathology, histology and clinical features. Gastric malt mucosaassociated lymphoid tissue lymphoma is a marginal zone lymphoma mzl of the stomach. The role of 18ffdgpetct in evaluating gastric malt lymphoma is still controversial. A lowgrade malignant lymphoma of the stomach associated with chronic infection by the bacterium h. Gastric mucosaassociated lymphoid tissue malt lymphoma responding to upfront treatment has an excellent outcome and no. In 45 patients treated in the past decade, the overall 5year survival was 86%, the causespecific survival was 95. Around 50% of gastric lymphomas are malt lymphomas.
It is also exemplifies the translation of biologic knowledge of a disease towards improved clinical practice. Gastric malt lymphoma is a model malignancy for examination of how neoplasia may arise from chronic inflammation. They represent 5% of gastric malignancies and show an apparently increasing incidence worldwide. Symptoms include loss of appetite, weight loss, indigestion, tiredness etc. Gastric mucosa associated lymphoid tissue lymphoma malt is a rare disease, 1 described for the first time in 1983 by isaacson and wright 2.
The main treatment of malt lymphoma include radiotherapy, chemotherapy, surgery or a combination. Furthermore, the optimal treatment of primary malt confined to the bladder remains to be defined. Sixtynine patients 26 female, 43 male with histologically. A 34yearold female with extranodal marginal zone lymphoma. May have coexisting gi or gu carcinomas am j surg pathol 1997. Malt lymphoma with concomitant dlbcl, not transformed to dlbcl over a 15year clinical course. Later the world health organisation renamed these tumours.
The immune activation and development of gastric mucosal lymphoid follicles follicular gastritis. Here, we report a case of a 65yearold female with primary malt lymphoma treated with definitive radiation therapy. Longterm followup results of no initial therapy for ocular adnexal malt lymphoma. Pdf the endoscopic findings of gastric malt lymphoma are variable, making its diagnosis very difficult. Nccn clinical practice guidelines in oncology nccn guidelines. It is distinct because it involves lymphoid proliferation in mucosaassociated lymphoid tissue malt rather than lymph nodes. Pylori and gastric malt lymphoma timeline 1982 marshall et al linked h. The stomach is the most frequent site of extranodal lymphoma. About 80% of malt lymphomas completely regress with eradication therapy. It is also the most common source of lymphomas in the gastrointestinal. Gastric mucosaassociated lymphoid tissue lymphoma and.
Infection with helicobacter pylori is an influential promoter of gastric lymphomagenesis initiation. Leukemic dissemination has only sporadically been described in malt lymphoma 4, 5. It has been significantly related to bone marrow infiltration. Gastric lymphoma accounts for only 3% of gastric cancers and 10% of lymphomas. The risk of spontaneous perforation of a gastric malt lymphoma is 410%.
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