Lapolla Pierfrancesco, Maiola Vincenza, Familiari Pietro, Tomei Gabriella, Gangemi Dominella, Ienzi Sara, Arcese Roberto, Palmieri Mauro, Relucenti Michela, Mingoli Andrea, Brachini Gioia, Nottola Stefania Annarita, D'Andrea Giancarlo, La Pira Biagia, Bruzzaniti Placido
Nuffield Department of Surgical Sciences, John Radcliffe Hospital, Headington, University of Oxford, Oxford OX1 2JD, UK.
Division of Neurosurgery, Fabrizio Spaziani Hospital, 03100 Frosinone, Italy.
J Clin Med. 2024 Aug 22;13(16):4959. doi: 10.3390/jcm13164959.
: Spinal cord lymphomas represent a minority of extranodal lymphomas and often pose diagnostic challenges by imitating primary spinal tumors or inflammatory/infective lesions. This paper presents a unique case of primary cauda equina lymphoma (PCEL) and conducts a comprehensive review to delineate the clinical and radiological characteristics of this rare entity. : A 74-year-old male presented with progressive paresthesia, motor weakness, and symptoms indicative of cauda equina syndrome. Neurological examination revealed paraparesis and sphincter dysfunction. Imaging studies initially suggested an intradural meningioma. However, surgical intervention revealed a diffuse large B-cell lymphoma infiltrating the cauda equina. : A systematic review of the pertinent literature identified 18 primary cauda equina lymphoma cases. These cases exhibited diverse clinical presentations, treatments, and outcomes. The mean age at diagnosis was 61.25 years for women and 50 years for men, with an average follow-up of 16.2 months. Notably, 35% of patients were alive at 18 months, highlighting the challenging prognosis associated with PCEL. : Primary spinal cord lymphomas, especially within the cauda equina, remain rare and diagnostically complex due to their nonspecific clinical manifestations. The review highlights the need to consider spinal cord lymphoma in patients with neurological symptoms, even without a history of systemic lymphoma. : Magnetic resonance imaging (MRI) serves as the primary diagnostic tool but lacks specificity. Histopathological examination remains the gold standard for definitive diagnosis. The review underscores the importance of timely biopsy in suspected cases to facilitate accurate diagnosis and appropriate management. : Current management involves biopsy and chemotherapy; however, optimal treatment strategies remain ambiguous due to the rarity of PCEL. Despite aggressive therapeutic interventions, prognosis remains poor, emphasizing the urgency for enhanced diagnostic and treatment modalities. : Primary cauda equina lymphoma poses diagnostic and therapeutic challenges, necessitating a high index of suspicion in patients with atypical spinal cord symptoms. Collaborative efforts between neurosurgical, oncological, and infectious diseases teams are imperative for timely diagnosis and management. Advancements in diagnostic precision and therapeutic options are crucial for improving patient outcomes.
脊髓淋巴瘤占结外淋巴瘤的少数,常因酷似原发性脊髓肿瘤或炎症/感染性病变而带来诊断挑战。本文报告了一例原发性马尾神经淋巴瘤(PCEL)的独特病例,并进行了全面综述,以阐明这一罕见疾病的临床和放射学特征。一名74岁男性出现进行性感觉异常、运动无力及提示马尾神经综合征的症状。神经系统检查发现双下肢轻瘫和括约肌功能障碍。影像学检查最初提示为硬膜内脑膜瘤。然而,手术干预发现为弥漫性大B细胞淋巴瘤浸润马尾神经。对相关文献的系统综述确定了18例原发性马尾神经淋巴瘤病例。这些病例呈现出多样的临床表现、治疗方法及预后。女性诊断时的平均年龄为61.25岁,男性为50岁,平均随访时间为16.2个月。值得注意的是,35%的患者在18个月时仍存活,这凸显了与原发性马尾神经淋巴瘤相关的预后挑战。原发性脊髓淋巴瘤,尤其是在马尾神经内的淋巴瘤,由于其非特异性临床表现,仍然罕见且诊断复杂。该综述强调,即使没有全身淋巴瘤病史,对于有神经症状的患者也需考虑脊髓淋巴瘤。磁共振成像(MRI)是主要的诊断工具,但缺乏特异性。组织病理学检查仍是明确诊断的金标准。该综述强调了在疑似病例中及时活检以促进准确诊断和适当管理的重要性。目前的治疗包括活检和化疗;然而,由于原发性马尾神经淋巴瘤罕见,最佳治疗策略仍不明确。尽管采取了积极的治疗干预措施,预后仍然很差,这凸显了改进诊断和治疗方式的紧迫性。原发性马尾神经淋巴瘤带来了诊断和治疗挑战,对于有非典型脊髓症状的患者需要高度怀疑。神经外科、肿瘤学和传染病团队之间的协作对于及时诊断和管理至关重要。提高诊断精度和治疗选择的进展对于改善患者预后至关重要。