Large B-cell Lymphoma With Interferon Regulatory Factor 4 Rearrangement Presenting as a Primary Central Nervous
Hannah Cutshall1, Vijay M Patel1, Philip E Ferguson2
1Department of Pathology, University of Arkansas for Medical Sciences, Arkansas, USA.
Insights
Large B-cell lymphoma with IRF4 rearrangement is a rare lymphoma subtype. This case report details the first known instance of this lymphoma presenting as a primary central nervous system lymphoma.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- The 2022 WHO Classification recognizes large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4r) as a distinct entity, often associated with a favorable prognosis.
- LBCL-IRF4r typically affects younger individuals and commonly presents in the head and neck region, particularly the Waldeyer ring.
Observation:
- This report describes a rare case of LBCL-IRF4r manifesting as a primary central nervous system (CNS) lymphoma.
- To date, primary CNS lymphoma with IRF4 rearrangement has not been documented in English medical literature.
Findings:
- While IRF4 rearrangement occurs in other hematolymphoid neoplasms, their distinct histology and immunophenotype differentiate them from LBCL-IRF4r.
- This case highlights the importance of recognizing unusual presentations of hematolymphoid neoplasms in the CNS.
Implications:
- Accurate diagnosis of LBCL-IRF4r is crucial for patient management due to its favorable prognosis and potential for targeted therapies.
- Pathologists must be aware of this entity for accurate diagnosis and classification of CNS hematolymphoid neoplasms.
Abstract:
Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 (IRF4) rearrangement (LBCL-IRF4r) is a rare type of lymphoma with an overall favorable prognosis, and has been included as a distinct entity in the 2022 revision of the World Health Organization Classification of Lymphoid Neoplasms. More common in pediatric/young adult populations and rare in older adults, it typically presents in the head and neck, specifically, the Waldeyer ring, and rarely in other sites. To our knowledge, it has not been reported as a primary central nervous system (CNS) lymphoma in the English language medical literature. Although IRF4 rearrangement can be seen in other hematolymphoid neoplasms, their histology and immunophenotype differ greatly. Familiarity with such unusual entities is important for pathologists in the work-up and diagnosis of hematolymphoid neoplasms of the CNS and is especially critical with this entity due to overall better prognosis and possible targeted treatments in the future. Here, we present a case of LBCL-IRF4r presenting as a primary CNS lymphoma in the brain.
