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Lymphoma - B cell neoplasms

Follicular lymphoma - GI


Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 9 January 2011, last major update January 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

General
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● Higher incidence of follicular lymphoma in duodenum compared to remainder of GI tract (Am J Surg Pathol 2000;24:688)

Epidemiology
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● Uncommon
● For primary disease, median age is 54 years
● May be slight female predominance

Etiology
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● Origin of duodenal tumors appears distinct from nodal follicular lymphomas, but more similar to MALT lymphoma (Mod Pathol 2009;22:940)

Clinical features
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● Rare; 1-3% of all B cell, non-Hodgkin lymphomas in GI tract, although frequency may be increasing due to detection with capsule endoscopy and double balloon endoscopy (Dig Dis Sci 2010;55:158)
● Typically low grade and low stage (Jpn J Clin Oncol 2008;38:547, Am J Surg Pathol 2002;26:216)
● Commonly arises in duodenum or terminal ileum or as small polypoid masses throughout bowel (lymphomatoid polyposis, which may also be due to mantle cell lymphoma)
● Transmural involvement is common (J Clin Gastroenterol 2008;42:997)
● Often nodal involvement, even with low stage disease
● Indolent with occasional relapses but no/rare deaths (Am J Surg Pathol 2002;26:216)
● Lugano staging is described at Table

Treatment
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● Treatment somewhat controversial since disease is rare and usually presents as low stage, but often multifocal (World J Gastroenterol 2010;16:5543)
● Rituximab with chemotherapy (cyclophosphamide, vincristine and prednisone, possibly doxorubicin)
● Also surgery, watchful waiting
● Possibly antibiotics (Ann Oncol 2008;19:254)
● Follow up with endoscopy (standard, capsule or double balloon)

Clinical images
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Endoscopic images

Gross description
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● Bowel wall thickening with multiple elevated nodules or plaques, possible bowel obstruction

Micro
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● Associated with lymphomatous polyposis
● Usually low grade and low stage; often transmural involvement
● Composed of neoplastic follicles with small cleaved cells
● Predominantly follicular growth pattern, occasionally mixed with diffuse, rarely entirely diffuse

Micro images
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Various images

Positive stains
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● CD10, CD20, bcl2; also CD75, CD79, bcl6

Negative stains
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● CD3, CD5, CD23, CD45RO, cyclin D1

Molecular
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● t(14;18) - the standard translocation for follicular lymphoma; associated with poorer clinical course (Cancer 2010 Dec 29 [Epub ahead of print])

End of Lymphoma - B cell neoplasms > Follicular lymphoma - GI


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