Department of Neurology, Yale School of Medicine, New Haven, USA.
Department of Neurology, Heidelberg University Hospital, Heidelberg University, Heidelberg, Germany.
J Neurooncol. 2024 Nov;170(2):309-317. doi: 10.1007/s11060-024-04792-2. Epub 2024 Aug 8.
Neurolymphomatosis refers to infiltration of the peripheral nervous system (PNS) by non-Hodgkin lymphoma (NHL). Diagnostic intervals in neurolymphomatosis and factors delaying diagnosis have not been evaluated. We therefore aimed to analyze diagnostic intervals in a large cohort.
The quality control database at Yale Cancer Center, Section of Neuro-Oncology, was searched for neurolymphomatosis cases diagnosed between 2001 and 2021. Univariate analyses were performed to identify parameters influencing diagnostic intervals.
We identified 22 neurolymphomatosis cases including 7 with primary and 15 with secondary disease, which occurred a median (range: 4-144) of 16 months after initial NHL diagnosis. Patients typically presented with painful polyneuropathy (73%), that was asymmetrical and rapidly progressive. Diagnosis was based on PNS biopsy (50%) or integration of neuroimaging findings (50%) with NHL history and diagnostic cerebrospinal fluid examinations. Median interval from symptom onset to diagnosis was 3 months (range: 1-12). Secondary neurolymphomatosis compared to primary disease (median 2 vs. 6 months, p = 0.02), and cases with rapidly-progressive asymmetrical neuropathy as opposed to other presentations (median 2 vs. 6 months; p < 0.001) were diagnosed earlier. Upfront conventional CT compared to other modalities (median 2 vs. 5 months p = 0.04) and nerve root localization as opposed to other disease sites (median 1.5 vs. 4 months; p = 0.04) delayed diagnosis.
NL type and localization, neuropathy course and distribution, and imaging modality selected for initial evaluation influence diagnostic intervals in neurolymphomatosis. Knowledge of this rare entity is critical for early suspicion, and diagnosis.
神经淋巴病是指非霍奇金淋巴瘤（NHL）对周围神经系统（PNS）的浸润。尚未评估神经淋巴病的诊断间隔以及导致诊断延迟的因素。因此，我们旨在分析大样本队列中的诊断间隔。
耶鲁癌症中心神经肿瘤学科的质量控制数据库中检索了 2001 年至 2021 年间诊断的神经淋巴病病例。进行单变量分析以确定影响诊断间隔的参数。
我们确定了 22 例神经淋巴病病例，其中 7 例为原发性，15 例为继发性，这些病例均在 NHL 初始诊断后中位数（范围：4-144）16 个月发生。患者通常表现为疼痛性多发性神经病（73%），表现为不对称性和快速进展性。诊断基于 PNS 活检（50%）或神经影像学发现（50%）与 NHL 病史和诊断性脑脊液检查相结合。从症状出现到诊断的中位时间为 3 个月（范围：1-12）。与原发性疾病相比，继发性神经淋巴病（中位数 2 个月 vs. 6 个月，p=0.02）和快速进展性不对称性神经病与其他表现相比（中位数 2 个月 vs. 6 个月；p<0.001）的诊断更早。与其他方式相比，常规 CT 作为初始评估手段（中位数 2 个月 vs. 5 个月，p=0.04）以及神经根定位与其他病变部位相比（中位数 1.5 个月 vs. 4 个月；p=0.04）会延迟诊断。
NL 类型和定位、神经病的过程和分布以及用于初始评估的成像方式会影响神经淋巴病的诊断间隔。对这种罕见疾病的了解对于早期怀疑和诊断至关重要。
