Abstract：
BACKGROUND: Spinal cord compression is a rare presentation of non-Hodgkin lymphoma (NHL) in children. We aimed to describe the prevalence, histological subtypes, clinical presentation, therapy, and outcome of those children in a population-based cohort. The chemotherapy regimen remained comparable over time.
METHODS: We retrospectively identified all children and adolescents with paresis as initial manifestations of the NHL between January 1990 and December 2020 from the NHL-BFM database. Characteristics, therapy, and outcome data were gathered from the database and patient files.
RESULTS: Fifty-seven of 4779 children (1.2%) presented with initial paresis due to spinal cord compression. The median age was 10.3 years (range, 3.1-18.0 years), and 33% were female. Initial symptoms were paresis/weakness (n = 50, 88%), back pain (n = 33, 58%), paresthesia (n = 23, 40%), and bladder dysfunction and/or constipation (n = 22, 39%), persisting for a median of 14 days before diagnosis. Subtype distribution was mature B-NHL (n = 41, 72%), precursor B-lymphoblastic lymphoma (LBL) (n = 12, 21%), anaplastic large cell lymphoma (ALCL) (n = 3, 5%), and T-LBL (n = 1, 2%). Initial emergency therapy included surgery (70%) and/or chemotherapy/steroids (63%). Five-year event-free survival and overall survival (80% ± 5% and 82% ± 5%, respectively) were comparable with all other NHL patients. Neurological symptoms persisted in approximately one-third of surviving patients at the last follow-up.
CONCLUSIONS: 1.2% of pediatric NHL patients presented with paresis from spinal cord compression mainly due to B-cell lymphomas. Neurological sequelae were observed in one-third of surviving patients.
METHODS: We retrospectively identified all children and adolescents with paresis as initial manifestations of the NHL between January 1990 and December 2020 from the NHL-BFM database. Characteristics, therapy, and outcome data were gathered from the database and patient files.
RESULTS: Fifty-seven of 4779 children (1.2%) presented with initial paresis due to spinal cord compression. The median age was 10.3 years (range, 3.1-18.0 years), and 33% were female. Initial symptoms were paresis/weakness (n = 50, 88%), back pain (n = 33, 58%), paresthesia (n = 23, 40%), and bladder dysfunction and/or constipation (n = 22, 39%), persisting for a median of 14 days before diagnosis. Subtype distribution was mature B-NHL (n = 41, 72%), precursor B-lymphoblastic lymphoma (LBL) (n = 12, 21%), anaplastic large cell lymphoma (ALCL) (n = 3, 5%), and T-LBL (n = 1, 2%). Initial emergency therapy included surgery (70%) and/or chemotherapy/steroids (63%). Five-year event-free survival and overall survival (80% ± 5% and 82% ± 5%, respectively) were comparable with all other NHL patients. Neurological symptoms persisted in approximately one-third of surviving patients at the last follow-up.
CONCLUSIONS: 1.2% of pediatric NHL patients presented with paresis from spinal cord compression mainly due to B-cell lymphomas. Neurological sequelae were observed in one-third of surviving patients.
摘要：
背景：脊髓压迫是儿童非霍奇金淋巴瘤（NHL）的罕见表现。我们的目的是描述患病率，组织学亚型,临床表现,治疗,以及基于人群的队列中这些儿童的结果。随着时间的推移，化疗方案保持可比性。
方法：我们从NHL-BFM数据库中回顾性地确定了1990年1月至2020年12月期间所有患有轻瘫的儿童和青少年为NHL的初始表现。特点,治疗,结果数据来自数据库和患者档案.
结果：4779名儿童中有57名（1.2％）因脊髓压迫而出现初始麻痹。中位年龄为10.3岁(范围，3.1-18.0年)，33%是女性。最初的症状是轻瘫/虚弱（n=50，88%），背痛（n=33，58%），感觉异常（n=23，40%），膀胱功能障碍和/或便秘（n=22，39%），在诊断前持续14天的中位数。亚型分布为成熟B-NHL（n=41，72%），前体B淋巴母细胞淋巴瘤（LBL）（n=12，21％），间变性大细胞淋巴瘤（ALCL）（n=3，5％），和T-LBL(n=1,2%)。最初的紧急治疗包括手术（70％）和/或化疗/类固醇（63％）。5年无事件生存率和总生存率（80%±5%和82%±5%，分别）与所有其他NHL患者具有可比性。在最后一次随访中，大约三分之一的存活患者的神经系统症状持续存在。
结论：1.2%的儿童NHL患者主要由于B细胞淋巴瘤而出现脊髓压迫导致的麻痹。在三分之一的存活患者中观察到神经系统后遗症。
方法：我们从NHL-BFM数据库中回顾性地确定了1990年1月至2020年12月期间所有患有轻瘫的儿童和青少年为NHL的初始表现。特点,治疗,结果数据来自数据库和患者档案.
结果：4779名儿童中有57名（1.2％）因脊髓压迫而出现初始麻痹。中位年龄为10.3岁(范围，3.1-18.0年)，33%是女性。最初的症状是轻瘫/虚弱（n=50，88%），背痛（n=33，58%），感觉异常（n=23，40%），膀胱功能障碍和/或便秘（n=22，39%），在诊断前持续14天的中位数。亚型分布为成熟B-NHL（n=41，72%），前体B淋巴母细胞淋巴瘤（LBL）（n=12，21％），间变性大细胞淋巴瘤（ALCL）（n=3，5％），和T-LBL(n=1,2%)。最初的紧急治疗包括手术（70％）和/或化疗/类固醇（63％）。5年无事件生存率和总生存率（80%±5%和82%±5%，分别）与所有其他NHL患者具有可比性。在最后一次随访中，大约三分之一的存活患者的神经系统症状持续存在。
结论：1.2%的儿童NHL患者主要由于B细胞淋巴瘤而出现脊髓压迫导致的麻痹。在三分之一的存活患者中观察到神经系统后遗症。
