神经淋巴瘤病（NL）是指周围神经系统（PNS）的淋巴瘤浸润。鉴于周围神经病变的广泛鉴别诊断、缺乏更大的队列以及随后无法获得预后因素或共识治疗，NL 的诊断和治疗具有挑战性。本研究旨在定义 NL 的特征和预后因素。使用 PubMed 和 Scopus 数据库对文献（2004-2023 年）进行系统回顾，并按照 PRISMA 指南进行报告。其中包括报告明确 NL 诊断病例的个体患者数据的研究。提取临床、放射学、病理学和结果信息。使用对数秩检验和Cox比例风险模型进行单变量和多变量生存分析。264项研究中总共积累了459个NL病例。 NL 是 197 名患者中恶性肿瘤（原发性 NL）的首发表现。 262 例已知非霍奇金淋巴瘤（继发性 NL）在中位 12 个月后出现 PNS 复发。 NL 主要表现为快速恶化、不对称的疼痛性多发性神经病。浸润结构包括周围神经（56%）、神经根（52%）、神经丛（33%）和脑神经（32%）。症状出现后平均 3 个月才做出诊断，原发性 NL 明显延迟。主要依靠PNS活检或FDG-PET，其诊断率较高（>90%）。尸检诊断很少见（3%）。大多数病例被归类为 B 细胞淋巴瘤 (90%)。 96% 的患者接受了肿瘤定向治疗，通常包括甲氨蝶呤或基于利妥昔单抗的联合化疗。中位总生存期为 18 个月。原发性 NL，无神经系统以外并发全身性疾病（风险比 [HR]：0.44；95% CI 0.25-0.78；p = 0.005），体能状态（ECOG <2，HR：0.30；95% CI 0.18-0.52；p < 0.0001）和基于利妥昔单抗的治疗（HR：0.46；95% CI 0.28-0.73；p = 0.001）在调整临床和社会人口统计学参数时被确定为多变量分析的有利预后标志物。神经影像学方式的进展，特别是 FDG- PET，促进 NL 诊断并提供高诊断率。然而，原发性 NL 的诊断延迟仍然很常见。基于利妥昔单抗的治疗可改善 NL 结局。研究结果可能有助于临床医生早期识别 NL、进行预后分层和治疗。

Neurolymphomatosis (NL) refers to lymphomatous infiltration of the peripheral nervous system (PNS). NL diagnosis and treatment are challenging given the broad differential diagnosis of peripheral neuropathy, the lack of larger cohorts, and the subsequent unavailability of prognostic factors or consensus therapy. This study aimed to define characteristics and prognostic factors of NL.A systematic review of the literature (2004-2023) was performed using PubMed and Scopus databases and reported following PRISMA guidelines. Studies reporting individual patient data on cases with definitive NL diagnosis were included. Clinical, radiologic, pathologic, and outcome information were extracted. Univariable and multivariable survival analyses were performed using log-rank tests and Cox proportional hazard models.A total of 459 NL cases from 264 studies were accumulated. NL was the first manifestation of malignancy (primary NL) in 197 patients. PNS relapse of known non-Hodgkin lymphoma (secondary NL) occurred in 262 cases after a median 12 months. NL predominantly presented with rapidly deteriorating, asymmetric painful polyneuropathy. Infiltrated structures included peripheral nerves (56%), nerve roots (52%), plexus (33%), and cranial nerves (32%). Diagnosis was established at a median of 3 months after symptom onset with substantial delays in primary NL. It mainly relied on PNS biopsy or FDG-PET, which carried high diagnostic yields (>90%). Postmortem diagnoses were rare (3%). Most cases were classified as B-cell (90%) lymphomas. Tumor-directed therapy was administered in 96% of patients and typically consisted of methotrexate or rituximab-based polychemotherapy. The median overall survival was 18 months. Primary NL without concurrent systemic disease outside the nervous system (hazard ratio [HR]: 0.44; 95% CI 0.25-0.78; p = 0.005), performance status (ECOG <2, HR: 0.30; 95% CI 0.18-0.52; p < 0.0001), and rituximab-based treatment (HR: 0.46; 95% CI 0.28-0.73; p = 0.001) were identified as favorable prognostic markers on multivariable analysis when adjusting for clinical and sociodemographic parameters.Advances in neuroimaging modalities, particularly FDG-PET, facilitate NL diagnosis and offer a high diagnostic yield. Yet, diagnostic delays in primary NL remain common. Rituximab-based therapy improves NL outcome. Findings may assist clinicians in early recognition, prognostic stratification, and treatment of NL.