N Valyraki, G Ahle, E Tabouret, R Houot, F Jardin, H Ghesquières, A Schmitt, J Paillassa, S Choquet, C Soussain, R Ursu, L Nichelli, M Legarff-Tavernier, B Mathon, L Rozenblum, K Mokhtari, K Hoang-Xuan, C Houillier
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Upon diagnosis, 10 patients could not walk, and seven had an indwelling urinary catheter. All of the patients had undergone MRI that revealed expansive lesion(s) with homogeneous contrast enhancement. Brain lesions were found in nine patients and CSF IL-10 was increased in 9 of 10 patients. The diagnosis of PCNSL was made based on brain biopsy (n=6), spinal cord biopsy or surgery (n=6) or cytologic analysis of CSF (n=4). All the patients were treated with high-dose methotrexate-based chemotherapy, followed by spinal cord irradiation (n=1) or autograft (n=4). The median PFS and OS were 29 and 51 months, respectively. Among the responders, 33% remained in a wheelchair, and only 25% regained the ability to walk without assistance.</p><p><strong>Conclusion: </strong>Considering the high risks associated with a spinal cord biopsy, the rarity of the disease and the lack of specificity of its clinicoradiological presentation, the diagnosis of spinal cord lymphoma is often delayed. Searching for other lymphomatous locations or assaying CSF IL-10 may be helpful in this disease, which can cause irreversible handicap.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.8000,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Primary central nervous system lymphoma of the spinal cord: A LOC network cohort study.\",\"authors\":\"N Valyraki, G Ahle, E Tabouret, R Houot, F Jardin, H Ghesquières, A Schmitt, J Paillassa, S Choquet, C Soussain, R Ursu, L Nichelli, M Legarff-Tavernier, B Mathon, L Rozenblum, K Mokhtari, K Hoang-Xuan, C Houillier\",\"doi\":\"10.1016/j.neurol.2024.11.003\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>Primary central nervous system lymphoma (PCNSL) mainly affects the brain (>90% cases); there are very little data pertaining to PCNSL involving the spinal cord.</p><p><strong>Methods: </strong>We retrospectively selected from the French LOC network database adult immunocompetent patients diagnosed with PCNSL involving the spinal cord between 2011 and 2022.</p><p><strong>Results: </strong>Of the 2043 patients records retrieved from the database, 16 patients (median age: 62.5 years, median Karnofsky performance status: 40) satisfied our study's selection criteria. 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引用次数: 0
摘要
目的:原发性中枢神经系统淋巴瘤(Primary central nervous system lymphoma, PCNSL)主要累及大脑(> ~ 90%);关于PCNSL涉及脊髓的数据很少。方法:我们回顾性地从法国LOC网络数据库中选择2011年至2022年间诊断为累及脊髓的PCNSL的成人免疫功能正常患者。结果:从数据库中检索到的2043例患者记录中,有16例患者(中位年龄:62.5岁,中位Karnofsky性能状态:40)满足我们的研究选择标准。中位诊断延迟为97天。确诊后,10名患者不能行走,7名患者有留置导尿管。所有患者均行MRI检查,病变呈扩张性,造影增强均匀。9例患者出现脑损伤,9例患者脑脊液IL-10升高。PCNSL的诊断基于脑活检(n=6)、脊髓活检或手术(n=6)或脑脊液细胞学分析(n=4)。所有患者均接受大剂量甲氨蝶呤化疗,随后进行脊髓照射(n=1)或自体移植(n=4)。中位PFS和OS分别为29个月和51个月。在应答者中,33%的人仍然坐在轮椅上,只有25%的人在没有帮助的情况下恢复了行走能力。结论:考虑到脊髓活检的高风险,罕见的疾病和缺乏特异性的临床放射学表现,脊髓淋巴瘤的诊断往往被推迟。寻找其他淋巴瘤位置或检测CSF IL-10可能对这种疾病有帮助,这种疾病可能导致不可逆转的障碍。
Primary central nervous system lymphoma of the spinal cord: A LOC network cohort study.
Purpose: Primary central nervous system lymphoma (PCNSL) mainly affects the brain (>90% cases); there are very little data pertaining to PCNSL involving the spinal cord.
Methods: We retrospectively selected from the French LOC network database adult immunocompetent patients diagnosed with PCNSL involving the spinal cord between 2011 and 2022.
Results: Of the 2043 patients records retrieved from the database, 16 patients (median age: 62.5 years, median Karnofsky performance status: 40) satisfied our study's selection criteria. The median diagnostic delay was 97 days. Upon diagnosis, 10 patients could not walk, and seven had an indwelling urinary catheter. All of the patients had undergone MRI that revealed expansive lesion(s) with homogeneous contrast enhancement. Brain lesions were found in nine patients and CSF IL-10 was increased in 9 of 10 patients. The diagnosis of PCNSL was made based on brain biopsy (n=6), spinal cord biopsy or surgery (n=6) or cytologic analysis of CSF (n=4). All the patients were treated with high-dose methotrexate-based chemotherapy, followed by spinal cord irradiation (n=1) or autograft (n=4). The median PFS and OS were 29 and 51 months, respectively. Among the responders, 33% remained in a wheelchair, and only 25% regained the ability to walk without assistance.
Conclusion: Considering the high risks associated with a spinal cord biopsy, the rarity of the disease and the lack of specificity of its clinicoradiological presentation, the diagnosis of spinal cord lymphoma is often delayed. Searching for other lymphomatous locations or assaying CSF IL-10 may be helpful in this disease, which can cause irreversible handicap.
期刊介绍:
The first issue of the Revue Neurologique, featuring an original article by Jean-Martin Charcot, was published on February 28th, 1893. Six years later, the French Society of Neurology (SFN) adopted this journal as its official publication in the year of its foundation, 1899.
The Revue Neurologique was published throughout the 20th century without interruption and is indexed in all international databases (including Current Contents, Pubmed, Scopus). Ten annual issues provide original peer-reviewed clinical and research articles, and review articles giving up-to-date insights in all areas of neurology. The Revue Neurologique also publishes guidelines and recommendations.
The Revue Neurologique publishes original articles, brief reports, general reviews, editorials, and letters to the editor as well as correspondence concerning articles previously published in the journal in the correspondence column.