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WHO Classification of Tumours
Diffuse large B-cell lymphoma (DLBCL), NOS


Diffuse large B-cell lymphoma (DLBCL) is the most frequent type of non-Hodgkin lymphoma, representing 30%-40% of cases. It consists of a diffuse proliferation of large neoplastic B-cells without significant admixture of centrocytes. It affects mainly adults, with a mean age of manifestation in the 7th decade. The majority of DLBCLs originates primarily in lymph nodes, but up to 40% are initially confined to extranodal sites
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008

. DLBCLs are aggressive but potentially curable with multiagent chemotherapy.

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 extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue+(MALT lymphoma)
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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