Oka Yuka, Yamada Shoki, Takeda Moe, Hashimoto Yuko
Department of Diagnostic Pathology, Fukushima Medical University School of Medicine, Fukushima, Japan.
J Clin Exp Hematop. 2025 Mar 28;65(1):28-39. doi: 10.3960/jslrt.24057. Epub 2025 Jan 30.
Primary central nervous system (CNS) lymphomas account for 1.9-3% of all brain tumors, with the majority being histologically classified as primary large B-cell lymphoma of the CNS (PCNS-LBCL). PCNS-LBCL is characterized by mature germinal center-exit B cells, and most cases of this phenotype are classified as activated B-cell-like phenotype according to gene expression profiling, or as non-germinal center B-cell-like phenotype (non-GCB type) according to Hans's algorithm. Genetically, PCNS-LBCL often shows mutations in MYD88 and CD79B, and is similar to MCD or C5 in genetic subtypes. Therefore, we here investigated the clinicopathological and molecular characteristics of primary CNS B-cell lymphomas (PCNSBLs), focusing on the differences in the frequency of MYD88 and CD79B mutations, as well as the prognosis between GCB and non-GCB types. Forty-two patients with PCNSBLs were included in this study, with 12 (28.6%) classified as GCB type and 30 (71.4%) as non-GCB type. There were no significant differences between the two types in gender, tumor location, or frequency of MYD88 and CD79B mutations. Even after consideration of the confounding of age and the presence of R-MPV therapy, the GCB type PCNSBLs tended to exhibit better prognosis. Overall survival tended to be better in those with the GCB/MYD88 mutation (-) group, followed by the GCB/MYD88 mutation (+) group, and the non-GCB type. We speculate that Hans's algorithm and MYD88 mutation may have potential prognostic value for PCNSBLs.
原发性中枢神经系统（CNS）淋巴瘤占所有脑肿瘤的1.9 - 3%，其中大多数在组织学上被分类为中枢神经系统原发性大B细胞淋巴瘤（PCNS - LBCL）。PCNS - LBCL的特征是成熟的生发中心输出B细胞，根据基因表达谱分析，这种表型的大多数病例被分类为活化B细胞样表型，或根据汉斯算法被分类为非生发中心B细胞样表型（非GCB型）。在基因方面，PCNS - LBCL经常显示MYD88和CD79B突变，并且在遗传亚型上与MCD或C5相似。因此，我们在此研究了原发性中枢神经系统B细胞淋巴瘤（PCNSBLs）的临床病理和分子特征，重点关注MYD88和CD79B突变频率的差异以及GCB型和非GCB型之间的预后情况。本研究纳入了42例PCNSBLs患者，其中12例（28.6%）被分类为GCB型，30例（71.4%）为非GCB型。这两种类型在性别、肿瘤位置或MYD88和CD79B突变频率方面没有显著差异。即使考虑到年龄和R - MPV治疗的混杂因素，GCB型PCNSBLs仍倾向于表现出更好的预后。总体生存率在GCB/MYD88突变（-）组中往往更好，其次是GCB/MYD88突变（+）组，然后是非GCB型。我们推测汉斯算法和MYD88突变可能对PCNSBLs具有潜在的预后价值。
