Cutshall Hannah, Patel Vijay M, Ferguson Philip E, Levy Rebecca A, Gokden Murat
Department of Pathology, University of Arkansas for Medical Sciences, Arkansas, USA.
Department of Pathology, Northwest Arkansas Pathology Associates, Arkansas, USA.
Cureus. 2025 May 8;17(5):e83753. doi: 10.7759/cureus.83753. eCollection 2025 May.
Large B-cell lymphoma (LBCL) with interferon regulatory factor 4 ()rearrangement (LBCL-r) 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 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-r presenting as a primary CNS lymphoma in the brain.
伴有干扰素调节因子4（IRF4）重排的大B细胞淋巴瘤（LBCL-r）是一种罕见的淋巴瘤，总体预后良好，已被纳入2022年版世界卫生组织淋巴肿瘤分类中的一个独立实体。它在儿童/年轻成人中更常见，在老年人中罕见，通常发生在头颈部，特别是瓦尔代尔环，很少发生在其他部位。据我们所知，英文医学文献中尚未报道其作为原发性中枢神经系统（CNS）淋巴瘤的情况。虽然IRF4重排在其他血液淋巴肿瘤中也可见，但它们的组织学和免疫表型差异很大。对于病理学家来说，熟悉这类不常见的实体对于中枢神经系统血液淋巴肿瘤的检查和诊断很重要，由于该实体总体预后较好且未来可能有靶向治疗，因此尤为关键。在此，我们报告一例以原发性脑CNS淋巴瘤形式出现的LBCL-r病例。
