Abstract

Purpose

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.

Methods

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.

Results

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).

Conclusion

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.

中文翻译：

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成功完成原发性中枢神经系统淋巴瘤初始治疗后的复发模式：一个病例系列。

摘要

目的

原发性中枢神经系统淋巴瘤（PCNSL）是非霍奇金淋巴瘤的一种亚型，累及脑，脊髓或软脑膜，无系统性疾病的证据。这种罕见的疾病约占所有原发性中枢神经系统（CNS）肿瘤的3％。基于甲氨蝶呤的治疗方案是该疾病的治疗标准，总生存期为14到55个月。明显完全缓解后可复发。我们试图了解复发患者的结局。

方法

这是IRB批准的对我们机构在2004年12月31日至2016年12月10日之间接受治疗的患者的调查。我们回顾性地将所有PCNSL病例确定为数据库注册表的一部分，并评估了这些病例的人口统计学信息，是否存在复发，复发部位，治疗方案和中位无复发生存期。

结果

该分析确定了44例经病理证实的PCNSL诊断的患者。诊断时的平均年龄为63.1岁（范围20-86，SD = 13.2岁）。在这44名患者中，有28名患者成功完成了初始治疗方案，而没有复发或毒性，需要改变治疗方法。11例患者发生了复发，其复发部位仅在中枢神经系统内（n = 5），仅在玻璃体液中（n = 1），仅在中枢神经系统外（n = 3）或中枢神经系统与中枢神经系统外的组合（ n = 2）。CNS以外的复发部位包括睾丸（n = 1），肺（n = 1），肾上腺（n = 1），肾/肾上腺（n = 1）和腹膜后（n = 1）。成功完成治疗后的中位无复发生存期为6.7年（95％CI 1.1，12.6）。

结论

成功的初始治疗后，PCNSL有复发的倾向，这种复发可能发生在CNS的内部和外部。对有PCNSL病史的非治疗患者要保持警惕，以指导早期诊断复发并开始积极治疗。