Rare gastrointestinal lymphomas: The endoscopic investigation

Table 1 Endoscopic features of rare gastrointestinal lymphomas according to two classification systems

Ref.	Wang et al[18]		Myung et al [17]
No. of patients	13		32
Endoscopic pattern	Mucosal - ulcerative	30.7%	Fungating	39%
	Mucosal - erosive	15.3%	Ulcerative	6%
	Polypoid	23%	Infiltrative	14%
	Massive	31%	Ulcerofungating	31%
			Ulceroinfiltrative	11%

Table 2 Table listing gastro-intestinal lymphoma with main gastro-intestinal organ involvement, typical presenting characteristics, typical immunophenotype and genotype and prognosis

Lymphoma histotype			Presenting characteristics	Main GI involvement	Main endoscopic pattern	Typical immunophenotype	Typical genotype	Prognosis
T and NK lymphomas	EATL		Celiac patients with abdominal pain and small intestine obstruction/perforation	Duodenum and jejunum	Multiple erosions and ulcers	CD3+, CD4-, CD8+/-, CD7+, CD5-, CD2+, TIA+, GrBPer+, CD30-/+, CD25-/+, CD56-/+, CD16-, CD57-, BCL6-, CD10-, EBV-, EMA-/+	TRB and TRG clonally rearranged +9q31.3 -16q12.1 +1q32.2-q41 +5q34-q35.2 +8q24 (MYC)	Poor
	PTCL		Poor performance status	Stomach and duodenum	Ulcerative	CD3+, CD4+, CD8-, CD7+, CD5+, CD2+, TIA-, GrBPer-, CD30-/+, CD25-, CD56-, CD16-, CD57-, BCL6-, CD10-, EBV-, EMA-	1TCR clonally rearranged +7q/+8q/+17q/+22q/-4q -5q/-6q/-9p/-10q/-12q/-13q	54% survival at five year Poor in case of high IPI score and stage III-IV disease
	Extranodal NK/T-cell lymphoma		Gastrointestinal bleeding and B symptoms	Small intestine	Multiple erosions and ulcers	cyCD3+, CD4-, CD8-/+, CD7-, CD5-, CD2+, TIA+, GrBPer+, CD30-, CD25-, CD56+, CD16-, CD57-, BCL6-, CD10-, EBV+, EMA-	TCR in germinal configuration No specific cytogenetic studies on this specific subtype	Poor especially if perforation occurs
	Adult T-cell leukemia/lymphoma		Abdominal pain, diarrhea, general fatigue, weight loss	No site preferences	Ulcers	CD3+, CD4+, CD8-, CD7-, CD5+, CD2+, TIA-, GrBPer-, CD30-/+, CD25++, CD56-, CD16-, CD57-, BCL6-, CD10-, EBV-, EMA-	TCR clonally rearranged Monoclonal integration of HTLV-1	Poor2 Good3
	Indolent lympho-prolipherative diseases of GI tract	T-LPD	Dyspepsia and mild diarrhea	Small intestine and colon	Unremarkable/friable or erythematous mucosa	CD3+, CD4-, CD8+, CD7+/-, CD5+/-, CD2+, TIA+/-, GrBPer-/+, CD30-, CD56-, EBV-	TCR-γ monoclonal	Indolent course
		NK-cell enteropathy	Vague symptoms (dyspepsia)	Stomach and small intestine	Lesions exhibit superficial ulceration, flat elevations with central depression and are associated with edema and local hemorrhage	cCD3+, CD4-, CD8-, CD7+, CD5-, TIA+, GrBPer+, CD56+, EBV-	TRC polyclonal or oligoclonal	Indolent course
Mantle cell lymphoma			Vague symptoms (dyspepsia)	Colon	Multiple polyposis, seldom with ulcerations	CD19+, CD20+, CD5+, CD10-, CD43+, sIg+, BCL6-, IRF4/MUM1-, Cyclin D1+	BCR rearranged t(11;14)(q13;q32)	Negative impact on prognosis
Follicular lymphoma			Vague symptoms (dyspepsia)	Second part of duodenum	Whitish polyps	CD19+, CD20+, CD5-, CD10+, CD43-, sIg+, BCL6+, IRF4/MUM1-/+, Cyclin D1-, α4β7+	BCR rearranged t( 14:18)(q32:q2 1)	Good
Extramedullary plasmacytoma			Alarm symptoms and obstruction	Stomach	Infiltrating mass	Plasmacells expressing CD79a+, CD38+, CD19-, CD138+, CD56+, usually CD20-	BCR rearranged t(11;14)(q32;q13)	Poor
PTLD			Alarm symptoms	Colon	Rubbery erythematous or ulcerated	Similar to DLBCL and Burkitt’s lymphoma CD19+, CD20+, CD5-/+, CD10-/+, CD43-/+, sIg+/-, BCL6-/+, IRF4/MUM1-/+, Cyclin D1-	Monoclonal BCR	Poor median survival 6 mo
Plasmablastic lymphoma			Alarm symptoms	Stomach	Large masses and exophitic processes	CD79a+, CD138+, CD38+, IRF4/MUM1, CD45-, CD20, PAX5-, CD56-	Clonal IgH chain gene rearranqement	Poor
Hodgkin’s lymphoma			Obstruction	Colon	Protruding mass	CD30+, CD15+, CD45-, CD20-, CD79a-, PAX5+, Ig-, OCT2-, BOB1-, CD3-, CD2-, CD5-, ALK-	Clonal immunoglobulin gene rearrangeme nts	Prognostic impact not known

¹Estrapolated from nodal counterpart but not explored in Primary GI lymphoma;

²ATLL acute and lymphoma types;

³ATLL chronic and smoldering types. TCR: T-cell receptor; BCR: B-cell receptor; EATL: Entheropathy–associated T-cell lymphoma; PTCL: Perypheral T-cell lymphoma; T-LPD: T-cell lymph-prolipferative disease; NK: Natural killer; DLBCL: Diffuse Large B-cell lymphoma; PTLD: Post-transplantation lymph-proliferative disease; GI: Gastro-intestinal.

Table 3 Prevalence of gastrointestinal lymphomas among transplant recipients according to transplanted organ

Transplant	Prevalence
Bone marrow	0.50%
Liver	1%-2%
Kidney	0.7%-4%
Heart	2%-10%
Small bowel	up to 30%

The data extracted from Heise[206], 2010.

Table 4 Reports of gastrointestinal plasmablastic lymphoma from 1998

Manuscript	Year	localization	Endoscopic appearance	HIV
Pruneri et al[230]	1998	Stomach	Large polypoid mass	-
Colomo et al[231]	2004	Anal region	Mass	+
Dong et al[232]	2005	GI tract	Not reported	+
		Small Intestine	Not reported	+
Tavora et al[228]	2006	Anal region	Not reported	+
		Anal region	Exophytic mass	+
Taddesse-Heath et al[233]	2010	Small intestine/colon (2 cases)	Not reported	+
Brahmania et al[234]	2011	Ano-rectal junction	Hypervascular cauliflower-like mass	-
Mihaljevic et al[235]	2012	Stomach	Not reported	-
Hashimoto et al[236]	2012	Stomach	Not reported	-
Chapman-Fredricks et al[229]	2012	Stomach	Not reported	+
Luria et al[237]	2014	Anal region	Mass	+
		Sigma	Mass	-
		Small bowel	Not reported	-
		Ileum	Not reported	-

HIV: Human immunodeficiency virus; GI: Gastrointestinal.