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7 - MALT and other marginal zone lymphomas
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- By Emanuele Zucca, Oncology Institute of Southern Switzerland (IOSI), Francesco Bertoni, Oncology Institute of Southern Switzerland (IOSI)
- Edited by Robert Marcus, King's College London, John W. Sweetenham, University of Utah, Michael E. Williams
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- Book:
- Lymphoma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 104-120
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Summary
Extranodal marginal zone B-cell lymphoma (MALT lymphoma)
Clinical features
Lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) comprises about 7-8% of all non-Hodgkin lymphomas (NHLs). It is a neoplasm of adults with a median age at presentation of about 60 years and with a slightly higher proportion of females than males. The presenting symptoms are essentially related to the primary location. Few patients present with elevated lactate dehydrogenase (LDH) or β2 microglobulin levels. Constitutional B-symptoms are extremely uncommon. MALT lymphoma usually remains localized for a prolonged period within the tissue of origin, but dissemination to multiple sites is not uncommon and has been reported in up to one-quarter of cases, with either synchronous or metachronous involvement of multiple mucosal sites or non-mucosal sites such as the bone marrow, the spleen, or the liver. Regional lymph nodes can also be involved. Bone marrow involvement is reported in up to 20% of cases.
The stomach is the commonest localization, representing about one-third of the cases. Other typical presentation sites include the salivary glands, the orbit, the thyroid, and the lung; the frequency of occurrence in different organs is shown in Table 7.1.
Within the stomach, MALT lymphoma is often multifocal, possibly explaining the reports of relapses in the gastric stump after surgical excision. Gastric MALT lymphoma can often disseminate to the small intestine and to the splenic marginal zone. Concomitant gastrointestinal (GI) and non-GI involvement can be detected in approximately 10% of cases.
9 - MALT lymphoma and other marginal zone lymphomas
- from Part II - LYMPHOMA SUBTYPES
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- By Emanuele Zucca, Lymphoma Unit Oncology, Institute of Southern Switzerland, Ospedale San Giovanni 6500, Bellinzona, Switzerland, Francesco Bertoni, Functional Genomics Unit, Laboratory of Experimental Oncology, Oncology, Institute of Southern Switzerland, 6500, Bellinzona, Switzerland, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
- Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
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- Book:
- Lymphoma: Pathology, Diagnosis and Treatment
- Published online:
- 05 March 2010
- Print publication:
- 21 June 2007, pp 126-140
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- Chapter
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Summary
EXTRANODAL MARGINAL ZONE B-CELL LYMPHOMA (MALT LYMPHOMA): CLINICAL FEATURES
Lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) comprises about 7–8% of all non-Hodgkin's lymphomas. It is a neoplasm of adults with a median age at presentation of about 60 years and with a slightly higher proportion of females than males. The presenting symptoms are essentially related to the primary location. Few patients present with elevated lactate dehydrogenase (LDH) or β2 microglobulin levels. Constitutional B symptoms are extremely uncommon. MALT lymphoma usually remains localized for a prolonged period within the tissue of origin, but dissemination to multiple sites is not uncommon and has been reported in up to one-quarter of cases, with either synchronous or metachronous involvement of multiple mucosal sites or non-mucosal sites such as bone marrow, spleen or liver. Regional lymph nodes can also be involved. Bone-marrow involvement is reported in up to 20% of cases. The stomach is the commonest localization, representing about one-third of the cases. Other typical presentation sites include the salivary glands, the orbit, the thyroid and the lung; the frequency at different organs is shown in Table 9.1.
Within the stomach, MALT lymphoma is often multifocal, possibly explaining the reports of relapses in the gastric stump after surgical excision. Gastric MALT lymphoma can often disseminate to the small intestine and to the splenic marginal zone. Concomitant GI and non-GI involvement can be detected in approximately 10% of cases. Disseminated disease appears to be more common in non-GI MALT lymphomas.