Vallejo Alexandra, González Iván D, Guerrero Lizcano Eduardo, Forero Oscar, Enciso Leonardo
Radiation Oncology, Instituto Nacional de Cancerología, Bogotá, COL.
Radiology, Instituto Nacional de Cancerología/Universidad Militar Nueva Granada, Bogotá, COL.
Cureus. 2021 Jun 18;13(6):e15735. doi: 10.7759/cureus.15735. eCollection 2021 Jun.
Primary central nervous system lymphomas (PCNSL) are extranodal non-Hodgkin's lymphomas (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. Primary leptomeningeal lymphoma (PLML), without parenchymal involvement, synchronous cerebrospinal, or systemic disease is rare. The estimated incidence of PLML is 7% of all PCNSL, which in turn accounts for about 2% of all primary brain tumors and 0.8% of all lymphomas. The incidence of PCNSL in Western countries is approximately five cases per million inhabitants per year, and less than 5% of all primary tumors of the central nervous system (CNS), although it is worth mentioning that the incidence seems to be increasing. The largest series of cases reported in the medical literature collect information from no more than nine patients; in these series, the median age at diagnosis is 57 years; in general, all patients present with cerebrospinal fluid alteration, and the median overall survival rate is close to eight months. With our case series, we aim at sharing the experience of four patients diagnosed and treated at the National Cancer Institute between 2010 and 2020, establishing a correlation of the clinical, imaging, and histopathological presentation, the response to treatment based on radiotherapy and chemotherapy, and the clinical outcomes reported in the medical records.
原发性中枢神经系统淋巴瘤(PCNSL)是局限于脑、软脑膜、眼或脊髓的结外非霍奇金淋巴瘤(NHL)。原发性软脑膜淋巴瘤(PLML),无实质受累、无同步脑脊液或全身性疾病,较为罕见。PLML的估计发病率占所有PCNSL的7%,而PCNSL又约占所有原发性脑肿瘤的2%和所有淋巴瘤的0.8%。西方国家PCNSL的发病率约为每年每百万居民5例,占中枢神经系统(CNS)所有原发性肿瘤的不到5%,不过值得一提的是,其发病率似乎在上升。医学文献中报道的最大病例系列收集的信息不超过9例患者;在这些系列中,诊断时的中位年龄为57岁;一般来说,所有患者都有脑脊液改变,中位总生存率接近8个月。通过我们的病例系列,我们旨在分享2010年至2020年期间在国家癌症研究所诊断和治疗的4例患者的经验,建立临床、影像学和组织病理学表现、基于放疗和化疗的治疗反应以及病历中报告的临床结果之间的关联。