Primary gastrointestinal lymphoma

Table 1 Expression of common markers and translocations in histological subtypes of gastrointestinal lymphomas

Type	CD5	CD10	CD19	CD20	CD22	CD23	CD43	CD79a	CD3	CD7	CD4	CD8	CD30	CD15	CD45RO	Additional features
Diffuse large B-cell lymphoma	-(+)	-(+)	+	+	+	-	-	+	-	-	-	-	-(+)	-	-(+)	Bcl-6+(-), Bcl-2+(-), t (14;18), t (3;14), t (8;14)
MALT lymphoma	-	-	+	+	+	-	-(+)	+	-	-	-	-	-	-	-(+)	t (11;18), t (14;18), t (1;14), t (3;14)
Follicular lymphoma	-	+(-)	+	+	+	-(+)	-	+	-	-	-	-	-(+)	-	-	Bcl-2+, Bcl-6+, t (14;18)
Burkitt lymphoma	-	+	+	+	+	-(+)	+	+	-	-	-	-	-	-	-	C-myc, t (8;14)
Mantle cell lymphoma	+	-	+	+	+	-	+	+	-	-	-	-	-	-	-	Cyclin D1+, t (11;14)
Peripheral T-cell lymphoma-unspecified	+(-)	-(+)	-	-	-	-	+(-)	-	+(-)	-(+)	+(-)	-(+)	-(+)	-(+)	-(+)	-
Extranodal NK/T cell lymphoma	-(+)	-	-	-	-	-	+	-	-(+)	+(-)	-(+)	-	-(+)	-(+)	-	EBV+, gains of 2q, 15q, 17q, 22q, losses of 6q, 8p, 11q, 12q, 13q
EATL	-	-	-	-	-	-	+/-	-	+	+	-(+)	+(-)	+(-)	-	+(-)	TIA1+, gains of 1q, 5q, 7q, 9q, losses of 8p, 9p, 13q
Hodgkin disease	+(-)	-	-	-(+)	-	-	-	-(+)	+(-)	-(+)	-	-	+(-)	+(-)	-(+)	Variable

+: ≥ 90% positive; +(-): > 50% positive; -(+): < 50% positive; -: < 10% positive cases. MALT: Mucosa-associated lymphoid tissue; EATL: Enteropathy-associated T-cell lymphoma; EBV: Epstein-Barr virus.

Table 2 Characteristic features of gastrointestinal lymphomas

Region	Age (yr)	GIL (%)	Sex	Predilection site	Etiological/risk factors	Presenting symptoms	Common pathological subtypes	Radiographic features
Oropharyngeal	> 50	-	M > F	Tonsil, nasopharynx1, base of tongue	EBV	Dysphagia, dyspnea, painless mass, ulcer, oral/hearing pain, B symptoms rare	DLBCL, EMZL/MALT, PTCL, FL, MCL, ENKL, HD	Lobular mass, ulcers
Esophagus	Variable	< 1	-	Mid and lower third	EBV, HIV	Dysphagia, odynophagia, weight loss, epigastric/chest pain, pneumonia, bleeding rare, B symptoms rare	DLBCL, MALT lymphoma, HD, MCL, T-cell lymphoma	Stricture, ulcerated mass, submucosal nodules, varicoid-like, achalasia-like, aneurysmal, fistula formation
Stomach	> 50	60-75	M > F	Antrum	H. pylori (MALT lymphoma), HTLV-1, HBV (DLBCL), EBV, HCV	Epigastric pain, dysphagia, nausea, vomiting, weight loss, abdominal mass, gastrointestinal bleeding, obstruction, perforation, B symptoms rare	DLBCL, MALT, PTCL	Ulcers, polypoid mass, thickened fold, mucosal nodularity, linitis plastica-like
Small intestine	Variable	20-30	Usually, M > F	Ileum, jejunum, duodenum, multiple sites	Celiac disease (EATL), C. jejuni (IPSID), EBV, HIV/AIDS	Abdominal pain, nausea, vomiting, weight loss, GI bleeding, obstructive symptoms, intussusceptions, perforation, diarrhea (in IPSID), B symptoms rare	DLBLCL, MALT, EATL, MCL, Burkitt lymphoma, FL, IPSID, PTCL, ENKL	Polypoid mass, multiple nodules, infiltrative form, ulcer, excavation, fistulization, extraluminal mass, mucosal thickening, strictures
Colon/rectum	50-70	6-12	M > F	Caecum, ascending colon, rectum	Celiac disease (EATL), EBV, H. pylori (MALT lymphoma)	Abdominal pain, weight loss, abdominal mass, lower GI bleeding, obstruction, perforation	DLBCL, MALT, EATL, MCL, PTCL, Burkitt lymphoma	Polypoid mass, ulcers, mucosal nodularity, cavitary mass, mucosal thickening, strictures, aneurysmal

¹Included here though usually not applicable. GIL: Gastrointestinal lymphoma; EBV: Epstein Barr virus; DLBCL: Diffuse large B cell lymphoma; EMZL: Extranodal marginal-zone lymphoma; PTCL: Peripheral T cell lymphoma; FL: Follicular lymphoma; EATL: Enteropathy-associated T-cell lymphoma; MCL: Mantle cell lymphoma; IPSID: Immunoproliferative small intestinal disease; HD: Hodgkin’s disease; HTLV-1: Human T-cell lymphotropic virus-1; HCV: Hepatitis C virus; HBV: Hepatitis B virus; H. pylori: Helicobacter pylori; C. jejuni: Campylobacter jejuni; HIV: Human immunodeficiency virus; AIDS: Acquired immune deficiency syndrome; ENKL: Extranodal NK/T-cell lymphoma.

Table 3 Paris staging system for primary gastrointestinal lymphomas

Stage	Gastrointestinal lymphomas
TX	Lymphoma extent not specified
T0	No evidence of lymphoma
T1	Lymphoma confined to the mucosa/submucosa
T1m	Lymphoma confined to mucosa
T1sm	Lymphoma confined to submucosa
T2	Lymphoma infiltrates muscularis propria or subserosa
T3	Lymphoma penetrates serosa (visceral peritoneum) without invasion of adjacent structures
T4	Lymphoma invades adjacent structures or organs
NX	Involvement of lymph nodes not assessed
N0	No evidence of lymph node involvement
N1	Involvement of regional lymph nodes
N2	Involvement of intra-abdominal lymph nodes beyond the regional area
N3	Spread to extra-abdominal lymph nodes
MX	Dissemination of lymphoma not assessed
M0	No evidence of extranodal dissemination
M1	Non-continuous involvement of separate site in gastrointestinal tract (e.g. stomach and rectum)
M2	Non-continuous involvement of other tissues (e.g. peritoneum, pleura) or organs (e.g. tonsils, parotid gland, ocular, adnexa, lung, liver, spleen, kidney, breast, etc.)
BX	Involvement of bone marrow not assessed
B0	No evidence of bone marrow involvement
B1	Lymphomatous infiltration of bone marrow
TNM	Clinical staging: status of tumor, node, metastasis, bone marrow
pTNMB	Histopathological staging: status of tumor, node metastasis, bone marrow
pN	The histological examination will ordinarily include six or more lymph nodes

Table 4 International prognostic index

Adverse risk factors

Age > 60 yr

≥ 2 extranodal sites

Ann arbor stage III-IV

Performance status ≥ 2 (ECOG)

High lactate dehydrogenase

Risk

Low (n = 0-1)

Low-intermediate (n = 2)

High-intermediate (n = 3)

High (n = 4-5)