Caruso Ludovico, Cannella Adriano, Sassara Giulia Maria, Rapisarda Antonio Maria, Passiatore Marco, Rovere Giuseppe, De Vitis Rocco
Department of Ageing, Neurosciences, Head-Neck and Orthopaedics Sciences, Hand Surgery Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, 00168 Rome, Italy.
Department of Orthopedic and Geriatric Sciences, Catholic University of the Sacred Heart, 00168 Rome, Italy.
Life (Basel). 2025 Aug 27;15(9):1357. doi: 10.3390/life15091357.
Primary lymphoma of peripheral nerves (PLPN) is a rare extranodal non-Hodgkin lymphoma that mimics benign nerve conditions, leading to diagnostic delays. This systematic review evaluates the clinical, radiological, and pathological features of PLPN, alongside diagnostic and therapeutic strategies.
A systematic search was conducted across PubMed, Scopus, and Web of Science, and identified 23 studies reporting 27 cases of PLPN. Data on demographics, clinical presentation, diagnostics, treatment, and outcomes were extracted and synthesized qualitatively due to study heterogeneity.
The sciatic nerve was most involved (48.15%), followed by the ulnar (18.5%) and radial nerves (18.5%). The median age at diagnosis was 58 years, with symptoms including motor deficits (88.9%), sensory disturbances (74.1%), and pain (70.4%). B-cell lymphomas accounted for 81.5% of cases, predominantly diffuse large B-cell lymphoma. MRI findings were non-specific; however, diffusion-weighted imaging (DWI) showed diagnostic potential. Treatments included combination therapies (51.9%), chemotherapy (25.9%), and surgery. Complete remission was achieved in 70.8%, with a 2-year survival rate of 83.3%.
PLPN is rare but likely underdiagnosed. Early recognition requires multidisciplinary collaboration, advanced imaging, and standardized protocols. Future research should focus on molecular characterization, diagnostic criteria, and treatment optimization to improve outcomes for this challenging condition.
周围神经原发性淋巴瘤（PLPN）是一种罕见的结外非霍奇金淋巴瘤，可模拟良性神经疾病，导致诊断延迟。本系统评价评估了PLPN的临床、影像学和病理特征，以及诊断和治疗策略。
在PubMed、Scopus和Web of Science上进行了系统检索，确定了23项报告27例PLPN病例的研究。由于研究的异质性，对人口统计学、临床表现、诊断、治疗和结果的数据进行了提取和定性综合分析。
坐骨神经受累最多（48.15%），其次是尺神经（18.5%）和桡神经（18.5%）。诊断时的中位年龄为58岁，症状包括运动功能障碍（88.9%）、感觉障碍（74.1%）和疼痛（70.4%）。B细胞淋巴瘤占病例的81.5%，主要为弥漫性大B细胞淋巴瘤。MRI表现无特异性；然而，扩散加权成像（DWI）显示出诊断潜力。治疗方法包括联合治疗（51.9%）、化疗（25.9%）和手术。70.8%的患者实现了完全缓解，2年生存率为83.3%。
PLPN罕见但可能诊断不足。早期识别需要多学科协作、先进的影像学检查和标准化方案。未来的研究应聚焦于分子特征、诊断标准和治疗优化，以改善这种具有挑战性疾病的治疗效果。
