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Gastrointestinal follicular lymphoma: Current knowledge and future challenges
Katsuyoshi Takata1,2, Tomoko Miyata-Takata2, Yasuharu Sato2
1Department of Lymphoid Cancer Research, British Columbia Cancer Research Centre, Vancouver, Canada.
Pathology International
|January 3, 2018
Summary
Gastrointestinal follicular lymphoma (GI-FL) is a distinct disease with indolent behavior, primarily affecting the duodenum. Its unique pathological and molecular features suggest separate management from nodal follicular lymphoma.
Area of Science:
- Gastroenterology
- Hematology
- Oncology
Background:
- Extranodal follicular lymphoma (FL) commonly involves the gastrointestinal (GI) tract.
- GI-FL exhibits an indolent clinical course, often localized without progression or transformation, distinguishing it from nodal FL.
Purpose of the Study:
- To characterize the clinicopathological and molecular features of GI-FL.
- To compare GI-FL with nodal FL and determine if separate management is warranted.
Main Methods:
- Review of macroscopic and microscopic findings in GI-FL cases.
- Analysis of pathological and molecular characteristics, including immunoglobulin heavy chain gene usage and B-cell immunophenotype.
- Comparison with nodal FL characteristics.
Main Results:
- The duodenum is the most frequent site of GI-FL, followed by the jejunum and ileum.
- Typical macroscopic appearance includes multiple white nodules.
- Histologically, GI-FL is predominantly low grade (Grade 1-2, >95%).
- Distinct features include immunoglobulin heavy chain deviation (VH4, VH5), memory B-cell immunophenotype, and molecular similarities to mucosa-associated lymphoid tissue lymphoma.
Conclusions:
- GI-FL possesses unique pathological and molecular characteristics compared to nodal FL.
- These distinct features support the consideration of GI-FL as a separate entity for management purposes.
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