Wilson Pamela E, Oleszek Joyce L, Clayton Gerald H
Department of Physical Medicine and Rehabilitation, B285, The Children's Hospital, 1056 E. 19th Avenue, Denver, Colorado 80218, USA.
J Spinal Cord Med. 2007;30 Suppl 1(Suppl 1):S15-20. doi: 10.1080/10790268.2007.11753963.
BACKGROUND/OBJECTIVE: Spinal cord tumors are a relatively rare diagnosis, accounting for 1% to 10% of all pediatric central nervous system tumors. Understanding the etiology and clinical outcomes of these tumors is therefore very important. This study presents detailed information regarding clinical presentation, histological findings, outcomes, functional assessment, and management of a series of patients with this diagnosis.
Retrospective, descriptive study.
Thirty-five children with a final diagnosis of spinal cord tumor or mass, excluding dysraphism.
Neurodevelopmental tumors (dermoid tumors, epidermoid tumors, and teratomas) were the most common tumor type (31%), followed by astrocytomas (29%) and neuroblastomas (14%). Other types included schwannomas, meningiomas, giant cell tumors, extradural cystic masses, leukemic-related masses, and masses related to neurofibromatosis. Mean age at diagnosis was 6.6 years (SD = 5.5 y) and did not vary significantly by tumor type except for children with neuroblastoma (mean = 0.4 y, SD = 0.5 y). More boys (57%) were identified in the series than girls (43%); however, there was no association between tumor type and sex. Presenting complaints of pain were noted in 57% and were localized to the back, neck, or extremities. Extremity weakness was reported as an initial presenting symptom in 46%. Three children had scoliosis as a presenting issue and 14 had gait abnormalities. Regardless of treatment modality, mobility was retained in 83% of children with or without gait aids. Neurogenic bowel and/or bladder were present in 23% of the population.
This study corroborates other studies indicating that intramedullary tumors are the predominant form of pediatric spinal cord tumor. This population, however, presented with an unusually large number of developmental tumors, contrary to several published studies. The disparity may be the result of this institution acting as a regional referral center, thus increasing the number of this type of patient. The population is too small to make any other conjecture. The predominance of astrocytomas and neuroblastomas among those patients with poor outcomes and the prevalence of developmental tumors suggest the need for broader investigation. Although, in general, spinal cord tumors are relatively rare, this preliminary study supports the need to further evaluate associations between tumor type, presenting symptoms, treatment, and functional outcome in children with spinal cord tumors.
背景/目的：脊髓肿瘤是一种相对罕见的诊断，占所有小儿中枢神经系统肿瘤的1%至10%。因此，了解这些肿瘤的病因和临床结果非常重要。本研究提供了一系列患有该诊断的患者的临床表现、组织学发现、结果、功能评估和治疗的详细信息。
回顾性描述性研究。
35名最终诊断为脊髓肿瘤或肿块的儿童，不包括脊柱裂。
神经发育性肿瘤（皮样囊肿、表皮样囊肿和畸胎瘤）是最常见的肿瘤类型（31%），其次是星形细胞瘤（29%）和神经母细胞瘤（14%）。其他类型包括神经鞘瘤、脑膜瘤、巨细胞瘤、硬膜外囊性肿块、白血病相关肿块以及与神经纤维瘤病相关的肿块。诊断时的平均年龄为6.6岁（标准差=5.5岁），除神经母细胞瘤患儿外（平均=0.4岁，标准差=0.5岁），不同肿瘤类型之间差异不显著。该系列中男孩（57%）比女孩（43%）多；然而，肿瘤类型与性别之间没有关联。57%的患者出现疼痛主诉，疼痛部位局限于背部、颈部或四肢。46%的患者报告肢体无力为初始症状。3名儿童以脊柱侧弯为首发问题，14名儿童有步态异常。无论采用何种治疗方式，83%的儿童无论有无步态辅助器具都保留了活动能力。23%的患者存在神经源性肠道和/或膀胱问题。
本研究证实了其他研究表明髓内肿瘤是小儿脊髓肿瘤的主要形式。然而，与一些已发表的研究相反，该人群中发育性肿瘤的数量异常多。这种差异可能是由于该机构作为区域转诊中心，从而增加了此类患者的数量。该人群规模太小，无法做出任何其他推测。星形细胞瘤和神经母细胞瘤在预后较差的患者中占主导地位以及发育性肿瘤的患病率表明需要进行更广泛的研究。虽然一般来说，脊髓肿瘤相对罕见，但这项初步研究支持进一步评估脊髓肿瘤患儿的肿瘤类型、症状表现、治疗和功能结果之间的关联的必要性。
