原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的浸润性弥漫性大B细胞淋巴瘤，限于中枢神经系统（CNS）。由于对CNS的免疫表型和PCNSL的肿瘤基因改变了解不足以及缺乏经验证的诊断生物标志物，推动翻译进展的努力受到了挫折。研究者们现在可以利用各种PCNSL动物模型来研究，这些模型具有与PCNSL形态和免疫表型相似的特点，然而，需要对这些动物模型进行认真而详细的重新检查，以澄清基因改变、迁移能力和免疫状态的差异。在本综述中，我们介绍了PCNSL肿瘤细胞的表型和基因型特征，编制了关于不同注射部位、细胞起源、接受动物和免疫状态的PCNSL临床前动物模型，并详细讨论了肿瘤细胞的趋化性和迁移能力以及PCNSL的新型治疗策略。我们预计选择合适的动物模型将作为一个明确定义的临床前系统，用于了解PCNSL的分子发病机制，从而加速新型有效治疗方法的发展。© 2023 Wiley Periodicals LLC.

Primary central nervous system lymphoma (PCNSL) is a rare and invasive diffuse large B cell lymphoma confined in central nervous system (CNS). The effort to press forward the translational progress has been frustrated by the insufficient understanding of immunophenotype of CNS and tumor genetic alterations of PCNSL, and the lack of validated diagnostic biomarkers. Researchers now have a variety of PCNSL animal models at their disposal that resemble the morphology and immunophenotype of PCNSL, however, a careful and detailed re-examination of these animal models is needed to clarify the differences in genetic alterations, migration capability, and immune status. In this review, we present the knowledge about the phenotypic and genotypic features of PCNSL tumor cells, and compile the preclinical animal models of PCNSL with regard to various injection sites, cell origins, recipient animals, and immune status, and elaborate on the tropism and migration of tumor cells and novel therapeutic strategies for PCNSL. We envisage that the selection of suitable animal models will serve as a well-defined preclinical system to understand the molecular pathogenesis of PCNSL, thereby galvanizing the development of novel and potent therapeutic approaches.© 2023 Wiley Periodicals LLC.