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WHO Classification of Tumours
Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)


Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises up to 8% of B-cell lymphomas and manifests predominantly in the stomach, less frequently in lung, skin, and ocular adnexa. It arises from the marginal zone of lymphoid tissues and is histologically characterized by small to medium sized morphologicall heterogeneous atypical lymphocytes.
In many cases of MALT lymphoma, there is a history of a chronic inflammatory disorder that results in accumulation of extra nodal lymphoid tissue. Risk factors include infectious organisms, e.g. H. pylori (gastric MALT lymphoma), Chlamydia psittaci (ocular adnexal MALT lymphoma) Campylobacter jejuni (IPSID) and Borrelia Burgdoferi (cutaneous MALT lymphoma) and autoimmune diseases.
Adults are predominantly affected. MALT lymphomas respond to radiotherapy and the clinical course is ususally favourable.

Other types of marginal zone lymphoma include:
Nodal marginal zone lymphoma (NMZL) and
Splenic B-cell marginal zone lymphoma (SMZL)

Primary lymphoma of the oesophagus is defined as an extranodal lymphoma arising in the oesophagus, with the bulk of disease localized to this site

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Isaacson PG (1999)
Gastrointestinal lymphomas of T- and B-cell types.
Mod Pathol 12: 151-8

. Contiguous lymph-node involvement may be seen, but peripheral and mediastinal lymph nodes, spleen or liver should not be affected and chest radioimaging and leukocyte count should be normal. Thus, by exclusion of any other site, the primary clinical presentation is in the oesophagus and therapy is directed at this site.
The oesophagus is a very uncommon primary site, accounting for < 1% of patients with lymphoma

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O?uzkurt L, Karabulut N, Cakmakci E, Besim A (1997)
Primary non-Hodgkin's lymphoma of the esophagus.
Abdom Imaging 22: 8-10

. Oesophageal involvement is more common as a secondary either from mediastinum, from nodal disease or from a primary gastric location. Less than 30 cases of bona fide primary oesophageal lymphoma have been described as single case reports or as small series of two cases. Patients are frequently male and usually aged > 50 years.
Most frequently, primary oesophageal lymphomas are diffuse large B-cell lymphoma (DLBCL)

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Sabljak P, Stojakov D, Bjelovic M, Mihaljevic B, Velickovic D, Ebrahimi K, Spica B, Pesko P (2008)
Primary esophageal diffuse large B-cell lymphoma: report of a case.
Surg Today 38: 647-50

or MALT lymphomas
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Hosaka S, Nakamura N, Akamatsu T, Fujisawa T, Ogiwara Y, Kiyosawa K, Hidaka E, Ota H, Katsuyama T, Inagaki H (2002)
A case of primary low grade mucosa associated lymphoid tissue (MALT) lymphoma of the oesophagus.
Gut 51: 281-4

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Kitamoto Y, Hasegawa M, Ishikawa H, Saito J, Yamakawa M, Kojima M, Nakano T (2003)
Mucosa-associated lymphoid tissue lymphoma of the esophagus: a case report.
J Clin Gastroenterol 36: 414-6

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Miyazaki T, Kato H, Masuda N, Nakajima M, Manda R, Fukuchi M, Tsukada K, Kojima M, Nakajima T, Kuwano H (2004)
Mucosa-associated lymphoid tissue lymphoma of the esophagus: case report and review of the literature.
Hepatogastroenterology 51: 750-3

. Immunodeficiency associated with infection with HIV
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Weeratunge CN, Bolivar HH, Anstead GM, Lu DH (2004)
Primary esophageal lymphoma: a diagnostic challenge in acquired immunodeficiency syndrome--two case reports and review.
South Med J 97: 383-7

and chronic immunosuppression
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Golioto M, McGrath K (2001)
Primary lymphoma of the esophagus in a chronically immunosuppressed patient with hepatitis C infection: case report and review of the literature.
Am J Med Sci 321: 203-5

appear to be risk factors.
Presentation of classical Hodgkin lymphoma has also been reported in the oesophagus, but is considered an extension of nodal disease

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Coppens E, El Nakadi I, Nagy N, Zalcman M (2003)
Primary Hodgkin's lymphoma of the esophagus.
AJR Am J Roentgenol 180: 1335-7

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Jones K, Pacella J, Wasty F (2007)
Hodgkin's disease of the oesophagus: a literature review.
Australas Radiol 51: 489-91

. Primary oesophageal T-cell lymphoma has been described, but is exceedingly rare
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Fujisawa S, Motomura S, Fujimaki K, Tanabe J, Tomita N, Hara M, Mohri H (1999)
Primary esophageal T cell lymphoma.
Leuk Lymphoma 33: 199-202

. Involvement of the oesophagus in widespread primary intestinal T-cell lymphoma has also been reported
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Ahn MJ, Park YW, Han D, Choi JH, Shin SJ, Yoon BC, Choi HS, Lee YY, Jung TJ, Choi IY, Park MH, Kim IS (2000)
A case of primary intestinal T-cell lymphoma involving entire gastrointestinal tract: esophagus to rectum.
Korean J Intern Med 15: 245-9