Abstract

Purpose

Primary central nervous system lymphoma (PCNSL) involving the hypothalamic–pituitary axis (H–P axis) is a rare intracranial neoplasm. We aimed to determine the unique characteristics and treatment outcomes of patients with PCNSL at the H–P axis and review the literature.

Methods

We retrospectively reviewed the electronic medical records of patients with PCNSL in our institute from 2000 to 2017. We analyzed patient characteristics, clinicopathologic features, imaging results, and treatment outcomes. Furthermore, we searched the PubMed database and gathered more cases from published studies to analyze patient treatment outcomes.

Results

A total of 488 patients were diagnosed with central nervous system lymphoma at our institute. Seven (1.4%) patients had H–P axis involvement, five had diffuse large B-cell lymphoma, and two had mucosa-associated lymphoid tissue lymphoma. All patients had anterior pituitary lobe dysfunction, and two had posterior lobe dysfunction. The median progression-free survival (PFS) for seven patients was 29.0 (range: 0.9–48.1) months, and the 3-year survival rate was 42.9%. Pooled analysis included 45 patients. The median PFS for these patients was 7.0 months (0.9–52.0), and the 2-year survival rate was 20%. Univariate and multivariate analyses revealed that the patients with visual field defects had better prognosis (p = 0.0153 and 0.043, respectively).

Conclusion

PCNSL at the H–P axis is associated with a higher rate of pituitary dysfunction than other parasellar pathologies. PCNSL at the H–P axis has a worse treatment outcome than PCNSL at other sites. However, visual field defect is related to a favorable prognosis in these patients.

中文翻译：

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涉及下丘脑-垂体轴的原发性中枢神经系统淋巴瘤：一个病例系列和汇总分析。

摘要

目的

涉及下丘脑-垂体轴（H-P轴）的原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的颅内肿瘤。我们旨在确定PCNSL患者在H–P轴上的独特特征和治疗结果，并复习文献。

方法

我们回顾性研究了我院2000年至2017年间PCNSL患者的电子病历。我们分析了患者特征，临床病理特征，影像学结果和治疗结果。此外，我们搜索了PubMed数据库，并从已发表的研究中收集了更多病例，以分析患者的治疗结果。

结果

我所共诊断出488例中枢神经系统淋巴瘤患者。7（1.4％）例患者有H–P轴受累，5例患有弥漫性大B细胞淋巴瘤，2例患有粘膜相关淋巴样淋巴瘤。所有患者均患有垂体前叶功能障碍，其中两名患有后叶功能障碍。7名患者的中位无进展生存期（PFS）为29.0个月（范围：0.9-48.1）个月，3年生存率为42.9％。汇总分析包括45名患者。这些患者的PFS中位数为7.0个月（0.9-52.0），2年生存率为20％。单因素和多因素分析显示，视野缺损的患者预后较好（ 分别为p = 0.0153和0.043）。

结论

HPS轴上的PCNSL与其他鞍旁病变相比，其垂体功能障碍的发生率更高。H-P轴的PCNSL的治疗效果比其他部位的PCNSL差。然而，视野缺损与这些患者的良好预后有关。