Department of Pharmacy, Duke University Medical Center, Durham, NC, USA.
Duke Cancer Institute, Duke University Medical Center, Durham, NC, USA.
J Neurooncol. 2020 Apr;147(2):477-483. doi: 10.1007/s11060-020-03446-3. Epub 2020 Mar 5.
Primary central nervous system lymphoma (PCNSL) is a subtype of non-Hodgkin's lymphoma that involves the brain, spinal cord, or leptomeninges, without evidence of systemic disease. This rare disease accounts for ~ 3% of all primary central nervous system (CNS) tumors. Methotrexate-based regimens are the standard of care for this disease with overall survival rates ranging from 14 to 55 months. Relapse after apparent complete remission can occur. We sought to understand the outcomes of patients who relapsed.
This is an IRB-approved investigation of patients treated at our institution between 12/31/2004 and 10/12/2016. We retrospectively identified all cases of PCNSL as part of a database registry and evaluated these cases for demographic information, absence or presence of relapse, location of relapse, treatment regimens, and median relapse-free survival.
This analysis identified 44 patients with a pathologically confirmed diagnosis of PCNSL. Mean age at diagnosis was 63.1 years (range 20-86, SD = 13.2 years). Of the 44 patients, 28 patients successfully completed an initial treatment regimen without recurrence or toxicity that required a change in therapy. Relapse occurred in 11 patients with the location of relapse being in the CNS only (n = 5), vitreous fluid only (n = 1), outside CNS only (n = 3), or a combination of CNS and outside of the CNS (n = 2). Sites of relapse outside of the CNS included testes (n = 1), lung (n = 1), adrenal gland (n = 1), kidney/adrenal gland (n = 1), and retroperitoneum (n = 1). Median relapse-free survival after successful completion of therapy was 6.7 years (95% CI 1.1, 12.6).
After successful initial treatment, PCNSL has a propensity to relapse, and this relapse can occur both inside and outside of the CNS. Vigilant monitoring of off-treatment patients with a history of PCNSL is necessary to guide early diagnosis of relapse and to initiate aggressive treatment.
原发性中枢神经系统淋巴瘤（PCNSL）是一种非霍奇金淋巴瘤，涉及脑、脊髓或软脑膜，而无全身疾病的证据。这种罕见的疾病占所有原发性中枢神经系统（CNS）肿瘤的~3%。甲氨蝶呤为基础的方案是这种疾病的标准治疗方法，总生存率从 14 到 55 个月不等。在明显完全缓解后可能会复发。我们试图了解复发患者的结局。
这是一项在我们机构进行的、经机构审查委员会批准的、对 2004 年 12 月 31 日至 2016 年 10 月 12 日期间治疗的患者进行的研究。我们回顾性地从数据库登记中确定了所有 PCNSL 病例，并对这些病例的人口统计学信息、有无复发、复发部位、治疗方案以及中位无复发生存期进行评估。
这项分析确定了 44 例经病理证实的 PCNSL 患者。诊断时的平均年龄为 63.1 岁（范围 20-86 岁，标准差=13.2 岁）。在 44 例患者中，28 例患者成功完成了初始治疗方案，无复发或毒性反应需要改变治疗。11 例患者发生复发，其中仅中枢神经系统复发（n=5），仅玻璃体液复发（n=1），仅中枢神经系统外复发（n=3），或中枢神经系统内外均有复发（n=2）。中枢神经系统外复发部位包括睾丸（n=1）、肺（n=1）、肾上腺（n=1）、肾脏/肾上腺（n=1）和腹膜后（n=1）。成功完成治疗后，中位无复发生存期为 6.7 年（95%CI 1.1，12.6）。
在成功的初始治疗后，PCNSL 有复发的倾向，这种复发可以发生在中枢神经系统内或外。对有 PCNSL 病史的停药患者进行警惕性监测，以指导早期诊断复发并启动积极治疗是必要的。
