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and HBeAg(-) patients - World Journal of Gastroenterology

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Ghimire P et al . Primary GI lymphoma<br />

Table 2 Characteristic features <strong>of</strong> gastrointestinal lymphomas<br />

Region Age (yr) GIL<br />

(%)<br />

complete remission after eradication therapy alone [28] . It has<br />

been shown that individuals with positive HBsAg have an<br />

increased risk <strong>of</strong> developing NHL [29] . It was reported that<br />

HBV plays a role in the development <strong>of</strong> B-cell NHL [30] . In<br />

contrast, primary gastric lymphoma with a T-cell phenotype<br />

is relatively rare, accounting for only 7% <strong>of</strong> primary<br />

gastric lymphomas in HTLV-1 infected endemic areas <strong>and</strong><br />

a relatively large number <strong>of</strong> such cases are secondary gastric<br />

involvement <strong>of</strong> adult T-cell leukemia. Primary gastric<br />

T-cell lymphoma without HTLV-1 infection is rare, <strong>and</strong><br />

sporadic cases have been reported [31] . The age <strong>of</strong> most gastric<br />

lymphoma <strong>patients</strong> is over 50 years with a relative predilection<br />

in males. Clinical symptoms <strong>of</strong> gastric lymphoma<br />

are nonspecific <strong>and</strong> indistinguishable from other benign<br />

<strong>and</strong> malignant conditions. The most common complaints<br />

<strong>of</strong> gastric lymphoma <strong>patients</strong> are epigastric pain, weight<br />

loss, nausea <strong>and</strong> vomiting. Occasionally, an abdominal<br />

mass is palpable. Lymphadenopathy is rare <strong>and</strong> its <strong>patients</strong><br />

<strong>of</strong>ten have no physical signs. Perforation, bleeding, or obstruction<br />

is very uncommon. Unlike nodal lymphoma, B<br />

constitutional symptom is not common.<br />

Although all histological kinds <strong>of</strong> nodal lymphoma<br />

can arise from the stomach, the majority <strong>of</strong> them are <strong>of</strong><br />

the B-cell origin, <strong>and</strong> MALT lymphoma <strong>and</strong> DLBCL ac-<br />

WJG|www.wjgnet.com<br />

Sex Predilection<br />

site<br />

Oropharyngeal > 50 - M > F Tonsil,<br />

nasopharynx 1 ,<br />

base <strong>of</strong> tongue<br />

Esophagus Variable < 1 - Mid <strong>and</strong> lower<br />

third<br />

Etiological/<br />

risk factors<br />

Stomach > 50 60-75 M > F Antrum H. pylori<br />

(MALT<br />

lymphoma),<br />

HTLV-1, HBV<br />

(DLBCL),<br />

EBV, HCV<br />

Small intestine Variable 20-30 Usually,<br />

M > F<br />

Ileum, jejunum,<br />

duodenum,<br />

multiple sites<br />

Colon/rectum 50-70 6-12 M > F Caecum,<br />

ascending<br />

colon, rectum<br />

Presenting symptoms Common pathological<br />

subtypes<br />

EBV Dysphagia, dyspnea,<br />

painless mass, ulcer, oral/<br />

hearing pain, B symptoms<br />

rare<br />

DLBCL, EMZL/<br />

MALT, PTCL, FL,<br />

MCL, ENKL, HD<br />

EBV, HIV Dysphagia, odynophagia, DLBCL, MALT<br />

weight loss, epigastric/ lymphoma, HD, MCL,<br />

chest pain, pneumonia,<br />

bleeding rare, B symptoms<br />

rare<br />

T-cell lymphoma<br />

Celiac disease<br />

(EATL), C.<br />

jejuni (IPSID),<br />

EBV, HIV/<br />

AIDS<br />

Celiac disease<br />

(EATL),<br />

EBV, H.<br />

pylori (MALT<br />

lymphoma)<br />

Epigastric pain, dysphagia,<br />

nausea, vomiting, weight<br />

loss, abdominal mass,<br />

gastrointestinal bleeding,<br />

obstruction, perforation, B<br />

symptoms rare<br />

Abdominal pain,<br />

nausea, vomiting,<br />

weight loss, GI bleeding,<br />

obstructive symptoms,<br />

intussusceptions,<br />

perforation, diarrhea (in<br />

IPSID), B symptoms rare<br />

Abdominal pain, weight<br />

loss, abdominal mass,<br />

lower GI bleeding,<br />

obstruction, perforation<br />

Radiographic features<br />

Lobular mass, ulcers<br />

Stricture, ulcerated<br />

mass, submucosal<br />

nodules, varicoidlike,<br />

achalasia-like,<br />

aneurysmal, fistula<br />

formation<br />

DLBCL, MALT, PTCL Ulcers, polypoid<br />

mass, thickened fold,<br />

mucosal nodularity,<br />

linitis plastica-like<br />

DLBLCL, MALT,<br />

EATL, MCL, Burkitt<br />

lymphoma, FL, IPSID,<br />

PTCL, ENKL<br />

DLBCL, MALT,<br />

EATL, MCL, PTCL,<br />

Burkitt lymphoma<br />

Polypoid mass,<br />

multiple nodules,<br />

infiltrative form,<br />

ulcer, excavation,<br />

fistulization,<br />

extraluminal mass,<br />

mucosal thickening,<br />

strictures<br />

Polypoid mass, ulcers,<br />

mucosal nodularity,<br />

cavitary mass, mucosal<br />

thickening, strictures,<br />

aneurysmal<br />

1 Included here though usually not applicable. GIL: Gastrointestinal lymphoma; EBV: Epstein Barr virus; DLBCL: Diffuse large B cell lymphoma; EMZL:<br />

Extranodal marginal-zone lymphoma; PTCL: Peripheral T cell lymphoma; FL: Follicular lymphoma; EATL: Enteropathy-associated T-cell lymphoma; MCL:<br />

Mantle cell lymphoma; IPSID: Immunoproliferative small intestinal disease; HD: Hodgkin’s disease; HTLV-1: Human T-cell lymphotropic virus-1; HCV:<br />

Hepatitis C virus; HBV: Hepatitis B virus; H. pylori: Helicobacter pylori; C. jejuni: Campylobacter jejuni; HIV: Human immunodeficiency virus; AIDS: Acquired<br />

immune deficiency syndrome; ENKL: Extranodal NK/T-cell lymphoma.<br />

count for over 90%. MALT lymphoma comprises up to<br />

50% <strong>of</strong> all primary lymphomas involving the stomach.<br />

Histologically, the most significant finding is the presence<br />

<strong>of</strong> a variable number <strong>of</strong> lymphoepithelial lesions defined<br />

by evident invasion <strong>and</strong> partial destruction <strong>of</strong> mucosal<br />

gl<strong>and</strong>s by the tumor cells. MALT lymphoma shows the<br />

immunophenotype <strong>of</strong> B cells in the normal marginal<br />

zone <strong>of</strong> spleen, Peyer’s patches <strong>and</strong> lymph nodes. The<br />

tumor B-cells can express the surface immunoglobulin<br />

<strong>and</strong> pan-B antigens (CD19, CD 20, <strong>and</strong> CD79a), the marginal<br />

zone-associated antigens (CD35 <strong>and</strong> CD21, <strong>and</strong> lack<br />

CD5, CD10, CD23) <strong>and</strong> cyclin D1. MALT lymphoma can<br />

be divided into H. pylori positive or negative based on the<br />

presence <strong>of</strong> H. pylori. H. pylori negative MALT lymphoma<br />

tends to have a higher positive rate for t (11;18) (q21;q21)<br />

translocation than H. pylori positive MALT lymphoma [32] .<br />

DLBCL, a heterogeneous group <strong>of</strong> tumors which are<br />

clinically, histologically, immunophenotypically, cytogenetically<br />

variable, can be divided into 3 subgroups, namely<br />

germinal-center B-cell-like, activated B-cell-like, <strong>and</strong> primary<br />

mediastinal DLBCL according to the gene expression<br />

patterns with each having a different prognostication.<br />

The most commonly seen translocations as mentioned<br />

earlier include t (14;18) (q32;q21) with BCL2-rearrange-<br />

700 February 14, 2011|Volume 17|Issue 6|

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