Yamada Seiji, Satou Akira, Tsuyuki Yuta, Iba Sachiko, Okumura Yuka, Ishikawa Eri, Ito Hideaki, Kogure Yasunori, Goto Naoe, Tanikawa Motoki, Shimada Kazuyuki, Tsukamoto Tetsuya, Karube Kennosuke, Yokoo Hideaki, Kataoka Keisuke, Tomita Akihiro, Mase Mitsuhito, Nakamura Shigeo
Division of Analytical Pathology, Oncology Innovation Center, Research Promotion Headquarters, Fujita Health University School of Medicine, Toyoake, Japan.
Department of Neurosurgery, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
Pathol Int. 2025 Jan;75(1):11-20. doi: 10.1111/pin.13496. Epub 2024 Dec 11.
CD5 expression is seen in 5%-10% of de novo diffuse large B-cell lymphomas (DLBCLs). Primary large B-cell lymphoma of the central nervous system (PCNS-LBCL) also exhibits CD5 expression in a minority of cases, however, clinicopathological and molecular features remain largely unclarified. Here we present the clinical, molecular, and pathological features of 11 CD5-positive () PCNS-LBCL cases, occupying 6.7% of all 165 PCNS-LBCLs diagnosed in our institutions. While CD5 systemic DLBCL has been recognized as a distinctive subgroup showing an aggressive clinical course, no obvious differences were found between CD5 and CD5-negative subgroups among the present CNS patients clinically. MYD88 p.L265P and CD79B p.Y196 mutations were detected in eight (73%) and seven (64%) cases, respectively, supporting previous reports. Notably, the microenvironmental immune cells were universally PD-L1/CD274-positive, and the higher levels tended to present favorable overall survival, as already evidenced in the PCNS-LBCL series. In contrast, neoplastic PD-L1/CD274 expression was undetectable in all cases. Indeed, no structural variations or copy number alterations involving PD-1 ligands were detected by targeted-capture sequencing and fluorescence in situ hybridization. While further studies are warranted, we may have confirmed similarity between PCNS-LBCLs and intravascular large B-cell lymphomas from a molecular standpoint.
5%-10%的原发性弥漫性大B细胞淋巴瘤（DLBCL）可见CD5表达。中枢神经系统原发性大B细胞淋巴瘤（PCNS-LBCL）在少数病例中也有CD5表达，然而，其临床病理和分子特征在很大程度上仍不明确。在此，我们呈现了11例CD5阳性的PCNS-LBCL病例的临床、分子和病理特征，占我们机构诊断的所有165例PCNS-LBCL的6.7%。虽然CD5阳性的系统性DLBCL已被认为是一个具有侵袭性临床病程的独特亚组，但在目前的中枢神经系统患者中，CD5阳性和阴性亚组之间在临床上未发现明显差异。分别在8例（73%）和7例（64%）病例中检测到MYD88 p.L265P和CD79B p.Y196突变，支持先前的报道。值得注意的是，微环境免疫细胞普遍为PD-L1/CD274阳性，且较高水平往往提示总体生存率良好，这在PCNS-LBCL系列中已有证据。相比之下，所有病例中均未检测到肿瘤性PD-L1/CD274表达。事实上，通过靶向捕获测序和荧光原位杂交未检测到涉及PD-1配体的结构变异或拷贝数改变。虽然有必要进一步研究，但我们可能已从分子角度证实了PCNS-LBCL与血管内大B细胞淋巴瘤之间的相似性。
