Lymphoproliferative Disorders of the Gastrointestinal Tract A Review and Pragmatic Guide to Diagnosis

Department of Pathology, Alta Bates Summit Medical Center, Berkeley, California 94705, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.84). 10/2011; 135(10):1283-97. DOI: 10.5858/arpa.2011-0145-RA
Source: PubMed


The gastrointestinal tract is the most common site of extranodal lymphomas. Although all histologic categories of malignant lymphoma develop in the gastrointestinal tract, large B-cell lymphomas predominate, followed by extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT) type; the latter is especially prevalent in stomach. The acceptance of extranodal marginal zone lymphoma of MALT type as a clinicopathologic entity has reduced the number of cases that formerly were interpreted as florid lymphoid hyperplasia ("pseudolymphoma"). Nonetheless, the distinction of lymphoid hyperplasia from a lymphoma of MALT type in small biopsy specimens remains problematic.
To assess the relevant morphologic, immunologic, molecular, and genetic properties of gastrointestinal lymphomas and to present a feasible tactic for diagnosis, expressly for small biopsy specimens.
Case-derived material and literature review using PubMed (National Library of Medicine).
Most gastrointestinal lymphomas are readily amenable to an unqualified diagnosis, primarily those cases consisting of monomorphic large cells whether of B- or T-cell lineage, including cases associated with enteropathy. Diagnosis for infiltrates dominated by small lymphocytes remains taxing, as the differential diagnosis embraces not only MALT lymphoma and lymphoid hyperplasia but also mantle cell lymphoma, follicular lymphoma, and chronic lymphocytic leukemia/small lymphocytic lymphoma. Adherence to strict morphologic criteria is the standard for diagnosis, but these criteria should be augmented by immunologic studies together with judicious use of molecular techniques to determine clonality. In establishing a diagnosis of gastric marginal zone lymphoma of MALT type, determination of t(11;18)(q21;q21) status may be required since this translocation has clinical ramifications.

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    • "[5] Enteropathy associated T-cell lymphoma (EATL) types I and II and extranodal NK/T-cell lymphoma, nasal type, are the most frequent types of lymphomas presenting with intestinal involvement.[6]–[8] Rarely, other types of PTCL such as ALK+ anaplastic large cell lymphoma and gamma-delta T-cell lymphoma can also arise in the GI tract or involve it secondarily.[9]–[11]. "
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