Cai Qingqing, Fang Yu, Young Ken H
Department of Medical Oncology, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, P.R China.
Department of Medical Oncology, Sun Yat-Sen University Cancer Center, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Guangzhou 510060, P.R China.
Transl Oncol. 2019 Mar;12(3):523-538. doi: 10.1016/j.tranon.2018.11.011. Epub 2019 Jan 4.
Primary central nervous system lymphoma (PCNSL) is a group of extranodal non-Hodgkin lymphoma that exhibits specific biological characteristics and clinical behavior, with an aggressive disease course and unsatisfactory patient outcomes. It is of great importance to identify aberrant genetic loci and important molecular pathways that might suggest potential targets for new therapeutics and provide prognostic information. In this review, we listed various genetic and epigenetic alterations that are involved in PCNSL pathogenesis. In the aspect of treatment, we summarized the related literatures and evaluated the efficacy of surgery, induction chemotherapy, radiotherapy, intrathecal chemotherapy, and autologous stem cell transplantation in PCNSL. We also proposed the possible new agents for recurrent and relapse PCNSL based on the result of recent clinical researches.
原发性中枢神经系统淋巴瘤（PCNSL）是一组结外非霍奇金淋巴瘤，具有特定的生物学特征和临床行为，疾病进展迅速，患者预后不佳。识别可能提示新治疗靶点并提供预后信息的异常基因位点和重要分子途径非常重要。在本综述中，我们列出了参与PCNSL发病机制的各种遗传和表观遗传改变。在治疗方面，我们总结了相关文献，并评估了手术、诱导化疗、放疗、鞘内化疗和自体干细胞移植在PCNSL中的疗效。我们还根据近期临床研究结果提出了针对复发和难治性PCNSL的可能新药物。
