Abstract：
OBJECTIVE: The aim of this study was to evaluate the clinical features of spinal schwannomas in patients with schwannomatosis and compare them with a large cohort of patients with solitary schwannomas and neurofibromatosis Type 2 (NF2).
METHODS: The study was a retrospective review of 831 patients with solitary schwannomas, 65 with schwannomatosis, and 102 with NF2. The clinical, radiographic, and pathological data were extracted with specific attention to the age at onset, location of tumors, initial symptoms, family history, and treatment outcome.
RESULTS: The male-to-female ratio of patients with schwannomatosis (72.3% vs 27.7%) was significantly higher than that of patients with solitary schwannomas (53.3% vs 46.7%) and NF2 (54.0% vs 46.0%), respectively (chi-square test, p = 0.012). The mean age at the first spinal schwannoma operation of patients with NF2 (24.7 ± 10.2 years) was significantly younger than that of patients with solitary schwannomas (44.8 ± 13.2 years) and schwannomatosis (44.4 ± 14.1 years; 1-way ANOVA, p < 0.001). The initial symptoms were similar among the 3 groups, with pain being the most common. The distribution of spinal tumors among the 3 groups was significantly different. The peak locations of spinal schwannomas in patients with solitary schwannomas were at C1-3 and T12-L3; in schwannomatosis, the peak location was at T12-L5. A preferred spinal location was not evident for intradural-extramedullary tumors in NF2. Only a slight prominence in the lumbar area could be observed. The patients in the 3 groups obtained similar benefits from the operation; the recovery rates in the patients with solitary schwannomas, NF2, and schwannomatosis were 50.1%, 38.0%, and 53.9%, respectively. The prognosis varied among spinal schwannomas in the patients with schwannomatosis. Up until the last date of follow-up, most patients with schwannomatosis (81.5%) had undergone a single spinal operation, but 12 patients (18.5%) had undergone multiple spinal operations. Patients with nonsegmental schwannomatosis or those with early onset disease seemed to have a poor prognosis; they were more likely to undergo multiple spinal operations. Small cauda equina nodules were common in patients with schwannomatosis (46.7%) and NF2 (86.9%); these small schwannomas appeared to have relatively static behavior. Two patients suspicious for schwannomatosis were diagnosed with NF2 with the detection of constitutional NF2 mutations; 1 had unilateral vestibular schwannoma, and the other had suspicious bilateral trigeminal schwannomas.
CONCLUSIONS: The clinical features of spinal schwannomas vary among patients with solitary schwannomas, NF2, and schwannomatosis. Spinal schwannomas of patients with NF2 appear to be more aggressive than those in patients with solitary schwannomas and schwannomatosis. Spinal schwannomas of schwannomatosis predominate in the lumbar area, and most of them can be treated successfully with surgery. The prognosis varies among spinal schwannomas of schwannomatosis; some patients may need multiple operations due to newly developed schwannomas. Sometimes, it is difficult to differentiate schwannomatosis from NF2 based on clinical manifestations. It is prudent to perform close follow-up examinations in patients with undetermined schwannomatosis and their offspring.
METHODS: The study was a retrospective review of 831 patients with solitary schwannomas, 65 with schwannomatosis, and 102 with NF2. The clinical, radiographic, and pathological data were extracted with specific attention to the age at onset, location of tumors, initial symptoms, family history, and treatment outcome.
RESULTS: The male-to-female ratio of patients with schwannomatosis (72.3% vs 27.7%) was significantly higher than that of patients with solitary schwannomas (53.3% vs 46.7%) and NF2 (54.0% vs 46.0%), respectively (chi-square test, p = 0.012). The mean age at the first spinal schwannoma operation of patients with NF2 (24.7 ± 10.2 years) was significantly younger than that of patients with solitary schwannomas (44.8 ± 13.2 years) and schwannomatosis (44.4 ± 14.1 years; 1-way ANOVA, p < 0.001). The initial symptoms were similar among the 3 groups, with pain being the most common. The distribution of spinal tumors among the 3 groups was significantly different. The peak locations of spinal schwannomas in patients with solitary schwannomas were at C1-3 and T12-L3; in schwannomatosis, the peak location was at T12-L5. A preferred spinal location was not evident for intradural-extramedullary tumors in NF2. Only a slight prominence in the lumbar area could be observed. The patients in the 3 groups obtained similar benefits from the operation; the recovery rates in the patients with solitary schwannomas, NF2, and schwannomatosis were 50.1%, 38.0%, and 53.9%, respectively. The prognosis varied among spinal schwannomas in the patients with schwannomatosis. Up until the last date of follow-up, most patients with schwannomatosis (81.5%) had undergone a single spinal operation, but 12 patients (18.5%) had undergone multiple spinal operations. Patients with nonsegmental schwannomatosis or those with early onset disease seemed to have a poor prognosis; they were more likely to undergo multiple spinal operations. Small cauda equina nodules were common in patients with schwannomatosis (46.7%) and NF2 (86.9%); these small schwannomas appeared to have relatively static behavior. Two patients suspicious for schwannomatosis were diagnosed with NF2 with the detection of constitutional NF2 mutations; 1 had unilateral vestibular schwannoma, and the other had suspicious bilateral trigeminal schwannomas.
CONCLUSIONS: The clinical features of spinal schwannomas vary among patients with solitary schwannomas, NF2, and schwannomatosis. Spinal schwannomas of patients with NF2 appear to be more aggressive than those in patients with solitary schwannomas and schwannomatosis. Spinal schwannomas of schwannomatosis predominate in the lumbar area, and most of them can be treated successfully with surgery. The prognosis varies among spinal schwannomas of schwannomatosis; some patients may need multiple operations due to newly developed schwannomas. Sometimes, it is difficult to differentiate schwannomatosis from NF2 based on clinical manifestations. It is prudent to perform close follow-up examinations in patients with undetermined schwannomatosis and their offspring.
摘要：
目的：本研究的目的是评估神经鞘瘤病患者的脊髓神经鞘瘤的临床特征，并将其与孤立性神经鞘瘤和2型神经纤维瘤病（NF2）患者的大型队列进行比较。
方法：该研究是对831例孤立性神经鞘瘤患者的回顾性研究，65患有神经鞘瘤病，和102与NF2。临床，射线照相,并提取病理数据，特别注意发病年龄，肿瘤的位置，最初的症状,家族史,和治疗结果。
结果：神经鞘瘤病患者的男女比例（72.3％vs27.7％）明显高于孤立性神经鞘瘤患者（53.3％vs46.7％）和NF2患者（54.0％vs46.0％），分别(卡方检验，p=0.012)。NF2患者首次脊柱神经鞘瘤手术的平均年龄（24.7±10.2岁）明显小于孤立性神经鞘瘤（44.8±13.2岁）和神经鞘瘤病（44.4±14.1岁；单向方差分析，p<0.001)。3组的初始症状相似，疼痛是最常见的。3组之间脊柱肿瘤的分布差异有统计学意义。孤立性神经鞘瘤患者的脊髓神经鞘瘤的峰值位置在C1-3和T12-L3；在神经鞘瘤病中，峰值位置在T12-L5。NF2中硬膜内髓外肿瘤的首选脊柱位置不明显。在腰部区域只能观察到轻微的突出。3组患者从手术中获得了相似的益处；孤立性神经鞘瘤患者的恢复率，NF2和神经鞘瘤病为50.1%,38.0%,和53.9%，分别。神经鞘瘤病患者的脊柱神经鞘瘤的预后各不相同。直到最后的随访日期，大多数神经鞘瘤病患者（81.5%）接受过一次脊柱手术，但12例患者(18.5%)接受了多次脊柱手术。非节段神经鞘瘤病或早发性疾病患者的预后较差；他们更有可能接受多次脊柱手术。神经鞘瘤病（46.7％）和NF2（86.9％）患者常见马尾小结节；这些小神经鞘瘤似乎具有相对静态的行为。两名怀疑神经鞘瘤病的患者被诊断为NF2，并检测到构成性NF2突变；1例患有单侧前庭神经鞘瘤，另一例有可疑的双侧三叉神经鞘瘤。
结论：孤立性神经鞘瘤患者的脊髓神经鞘瘤的临床特征各不相同，NF2和神经鞘瘤病。NF2患者的脊髓神经鞘瘤似乎比孤立性神经鞘瘤和神经鞘瘤病患者更具侵袭性。神经鞘瘤病的脊柱神经鞘瘤在腰椎区占主导地位，他们中的大多数可以通过手术成功治疗。神经鞘瘤病的脊柱神经鞘瘤的预后各不相同；由于新发展的神经鞘瘤，一些患者可能需要多次手术。有时候,根据临床表现很难区分神经鞘瘤病和NF2。对未确定的神经鞘瘤病患者及其后代进行密切随访检查是谨慎的。
方法：该研究是对831例孤立性神经鞘瘤患者的回顾性研究，65患有神经鞘瘤病，和102与NF2。临床，射线照相,并提取病理数据，特别注意发病年龄，肿瘤的位置，最初的症状,家族史,和治疗结果。
结果：神经鞘瘤病患者的男女比例（72.3％vs27.7％）明显高于孤立性神经鞘瘤患者（53.3％vs46.7％）和NF2患者（54.0％vs46.0％），分别(卡方检验，p=0.012)。NF2患者首次脊柱神经鞘瘤手术的平均年龄（24.7±10.2岁）明显小于孤立性神经鞘瘤（44.8±13.2岁）和神经鞘瘤病（44.4±14.1岁；单向方差分析，p<0.001)。3组的初始症状相似，疼痛是最常见的。3组之间脊柱肿瘤的分布差异有统计学意义。孤立性神经鞘瘤患者的脊髓神经鞘瘤的峰值位置在C1-3和T12-L3；在神经鞘瘤病中，峰值位置在T12-L5。NF2中硬膜内髓外肿瘤的首选脊柱位置不明显。在腰部区域只能观察到轻微的突出。3组患者从手术中获得了相似的益处；孤立性神经鞘瘤患者的恢复率，NF2和神经鞘瘤病为50.1%,38.0%,和53.9%，分别。神经鞘瘤病患者的脊柱神经鞘瘤的预后各不相同。直到最后的随访日期，大多数神经鞘瘤病患者（81.5%）接受过一次脊柱手术，但12例患者(18.5%)接受了多次脊柱手术。非节段神经鞘瘤病或早发性疾病患者的预后较差；他们更有可能接受多次脊柱手术。神经鞘瘤病（46.7％）和NF2（86.9％）患者常见马尾小结节；这些小神经鞘瘤似乎具有相对静态的行为。两名怀疑神经鞘瘤病的患者被诊断为NF2，并检测到构成性NF2突变；1例患有单侧前庭神经鞘瘤，另一例有可疑的双侧三叉神经鞘瘤。
结论：孤立性神经鞘瘤患者的脊髓神经鞘瘤的临床特征各不相同，NF2和神经鞘瘤病。NF2患者的脊髓神经鞘瘤似乎比孤立性神经鞘瘤和神经鞘瘤病患者更具侵袭性。神经鞘瘤病的脊柱神经鞘瘤在腰椎区占主导地位，他们中的大多数可以通过手术成功治疗。神经鞘瘤病的脊柱神经鞘瘤的预后各不相同；由于新发展的神经鞘瘤，一些患者可能需要多次手术。有时候,根据临床表现很难区分神经鞘瘤病和NF2。对未确定的神经鞘瘤病患者及其后代进行密切随访检查是谨慎的。
