目的:神经淋巴瘤病(NL)的特征是周围神经系统的淋巴瘤浸润,表现为淋巴瘤的初始表现(原发性NL[PNL])或已知淋巴瘤的复发(继发性NL[SNL])。本报告详细介绍并比较了这两组的神经临床病理特征。
方法:这项回顾性研究是对1992年1月1日至2020年6月31日之间经病理证实为神经NL的患者进行的。患者临床特征,神经系统检查,影像学检查,EMG,收集神经活检数据,分析,并在PNL和SNL之间进行了比较。
结果:共确认58例患者(34个PNL和24个SNL)。与SNL的5.5个月相比,PNL从神经系统症状发作到诊断的时间更长,为18.5个月(p=0.01)。两组患者的神经系统症状相似,主要包括感觉丧失(98%)。严重疼痛(76%),和不对称的弱点(76%)。观察到广泛的EMG证实的不同神经病变模式,但与PNL(n=1,p=0.01)相比,SNL患者的单神经病变(n=8)数量增加.与氟脱氧葡萄糖(FDG)-PETCT成像研究(60%)相比,MRI研究检测到NL的频率更高(86%)(p=0.007)。神经活检显示B细胞淋巴瘤(PNLn=32,SNLn=22),其次是T细胞淋巴瘤(PNLn=2,SNLn=2),在SNL和PNL中,两组的脱髓鞘增加,轴突变性增加(p=0.01)和多灶性有髓纤维丢失(p=0.04)显着。与PNL相比,鉴定SNL导致患者治疗改变,但预后更差(p=0.025)。
结论:虽然PNL和SNL都是主要的疼痛和不对称神经病,在肌电图和神经活检上有轴突和脱髓鞘特征,SNL的表现与PNL略有不同,不对称通常在MRI上比FDG-PET/CT更好地检测到单神经病变。SNL的局灶性模式可能是逃避初始化疗的残留癌细胞的结果,它不穿过血液神经屏障,这些细胞后来会复发并导致暴发性疾病。虽然仍然导致预后较差,识别SNL很重要,因为这改变了每个SNL病例的治疗和管理。
OBJECTIVE: Neurolymphomatosis (NL) is characterized by lymphomatous infiltration of the peripheral nervous system presenting as the initial manifestation of a lymphoma (primary NL [PNL]) or in relapse of a known lymphoma (secondary NL [SNL]). This report details and compares the neurologic clinicopathologic characteristics of these 2 groups.
METHODS: This retrospective study was performed on patients diagnosed with pathologically confirmed NL in nerve between January 1, 1992, and June 31, 2020. Patient clinical characteristics, neurologic examination, imaging studies, EMG, and nerve biopsy data were collected, analyzed, and compared between PNL and SNL.
RESULTS: A total of 58 patients were identified (34 PNL and 24 SNL). Time from neurologic symptom onset to diagnosis was longer in PNL at 18.5 months compared with 5.5 months in SNL (p = 0.01). Neurologic symptoms were similar in both patient groups and included primarily sensory loss (98%), severe pain (76%), and asymmetric weakness (76%). A wide spectrum of EMG-confirmed different neuropathy patterns were observed, but patients with SNL had increased numbers of mononeuropathies (n = 8) compared with PNL (n = 1, p = 0.01). MRI studies detected NL more frequently (86%) compared with fluorodeoxyglucose (FDG)-PET CT imaging studies (60%) (p = 0.007). Nerve biopsies revealed B-cell lymphoma (PNL n = 32, SNL n = 22), followed by T-cell lymphoma (PNL n = 2, SNL n = 2), with increased demyelination in both groups and increased axonal degeneration (p = 0.01) and multifocal myelinated fiber loss (p = 0.04) significant in SNL vs PNL. Identifying SNL resulted in patient treatment modifications but a worse prognosis compared with PNL (p = 0.025).
CONCLUSIONS: While PNL and SNL are both primarily painful and asymmetric neuropathies with axonal and demyelinating features on EMG and nerve biopsy, SNL presents somewhat differently than PNL with fulminant, asymmetric often mononeuropathies better detected on MRI than FDG-PET/CT. The focal pattern of SNL is likely a result of residual cancer cells that evaded initial chemotherapy, which does not cross the blood-nerve barrier, and these cells can later recur and result in fulminant disease. Although still resulting in a poorer prognosis, identifying SNL is important because this changed treatment and management in every SNL case.