Peruzzi Pierpaolo, Ray-Chaudhuri Abhik, Slone Wayne H, Mekhjian Hagop S, Porcu Pierluigi, Chiocca E Antonio
Department of Neurological Surgery, The Ohio State University College of Medicine, Columbus, Ohio 43210, USA.
J Neurosurg. 2009 Aug;111(2):247-51. doi: 10.3171/2008.11.JNS08291.
Neurolymphomatosis, the infiltration of the peripheral nervous system (PNS) by malignant lymphatic cells, is a rare condition whose prognosis and treatment are not fully characterized. The authors report the case of a 69-year-old, previously healthy man who had a 1-month history of progressive pain in his right arm and associated weakness of several muscles of the right upper extremity when they first examined him. Initial MR imaging of the right brachial plexus showed no abnormalities, but over 3 months, symptoms gradually progressed to almost complete plegia of his right upper extremity. Subsequent MR imaging of his right brachial plexus showed an enhancing mass of the posterior cord of the plexus that encroached on the other cords. Positron emission tomography confirmed the presence of a hypermetabolic lesion in the right axillary region and also detected an asymptomatic hot spot in the gastric wall. Biopsy of the gastric lesion demonstrated a CD20+, diffuse large B-cell lymphoma that was immunohistochemically positive for BCL-6 and negative for p16. The patient underwent 6 cycles of dose-adjusted etoposide-vincristine-doxorubicin-cyclophosphamide-prednisone (EPOCH) and rituximab, intermixed with 3 cycles of high-dose intravenous and intrathecal methotrexate, and followed by 6 monthly doses of rituximab for consolidation. Follow-up MR imaging and PET of the plexus showed complete radiological response after 3 months of treatment, as demonstrated by normalization of brachial plexus caliber, contrast enhancement, and metabolic activity. Twenty-eight months after symptom onset and 20 months after beginning therapy, the patient was disease-free, had recovered most upper extremity neurological function, and had only minimal remaining weakness of the right wrist and finger extension.
神经淋巴瘤是指恶性淋巴细胞浸润周围神经系统（PNS），是一种罕见疾病，其预后和治疗尚未完全明确。作者报告了一例69岁既往健康男性病例，初诊时患者有1个月的右臂进行性疼痛病史，并伴有右上肢几块肌肉的相关无力症状。右侧臂丛神经的初始磁共振成像（MR）未显示异常，但在3个月内，症状逐渐发展为右上肢几乎完全瘫痪。随后对其右侧臂丛神经进行的MR成像显示，神经丛后束有一个强化肿块，侵犯了其他神经束。正电子发射断层扫描（PET）证实右侧腋窝区域存在高代谢病变，同时在胃壁发现一个无症状热点。胃病变活检显示为CD20阳性的弥漫性大B细胞淋巴瘤，免疫组化检测BCL-6呈阳性，p16呈阴性。患者接受了6个周期的剂量调整依托泊苷-长春新碱-阿霉素-环磷酰胺-泼尼松（EPOCH）和利妥昔单抗治疗，期间穿插3个周期的大剂量静脉和鞘内甲氨蝶呤治疗，随后每月给予6剂利妥昔单抗进行巩固治疗。对神经丛的随访MR成像和PET显示，治疗3个月后放射学上完全缓解，表现为臂丛神经管径、对比增强和代谢活性恢复正常。症状出现28个月后及开始治疗20个月后，患者无疾病，右上肢大部分神经功能已恢复，仅右腕和手指伸展仍有轻微无力。
