Nawabi Noah L A, Chalif Joshua I, Renauld Sophia, Mehta Neel H, Jha Rohan, Gerstl Jakob V E, Chalif Eric, Gupta Saksham, Chi John H
1Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
2College of Medicine, Medical University of South Carolina, Charleston, South Carolina.
J Neurosurg Spine. 2024 Nov 15;42(2):238-247. doi: 10.3171/2024.7.SPINE24607. Print 2025 Feb 1.
Spinal meningioma (SM) is a pathology with an estimated incidence of nearly 1000 diagnoses per year in the United States and presents in 20% of patients with neurofibromatosis type 2 (NF2). This multi-institutional retrospective cohort study aimed to assess clinical outcomes for patients with SM who underwent surgery between 1998 and 2020 with stratification by NF2 mutation status.
Medical records were reviewed retrospectively to collect data on patient demographics, clinical presentation, tumor characteristics, treatment, and outcomes. Analyses were done to determine radiographic predictors of gross-total resection (GTR) and tumor recurrence, to assess radiographic characteristics of NF2-associated tumors, and to determine progression-free survival between groups.
A total of 166 patients who received surgery for SM during the study period were included, of whom 133 were women (80%). Fifteen (9%) patients had a concurrent NF2 diagnosis. The mean age at surgery was 58 (SD 18) years. The mean presenting Karnofsky Performance Status score was 76 (SD 11), and the most common presenting symptoms were sensation changes (60%) and weakness (59%). Most tumors were in the thoracic spine (72%). GTR was achieved in 154 cases (93%). Eight patients with subtotal resection were treated with radiation therapy, and none received chemotherapy. Eighteen patients (11%) experienced radiographic recurrence of disease following surgery, with a mean time to recurrence of 4.2 years. NF2 patients were diagnosed at a significantly earlier mean age (33.3 [SD 15.4] years) compared with other patients. NF2 patients experienced progression at a significantly higher rate than other patients (40%), and in less time (mean 2.8 [SD 3.7] years). Radiographic characteristics, including tumor volume, T2 cord edema, dural tail sign, and calcification, were similar between NF2 and non-NF2 patients, between patients who underwent gross-total versus subtotal resection, and between patients who experienced tumor recurrence and those who did not.
In this study of 166 surgically treated patients with SM, patients with NF2 presented earlier, experienced earlier progression, and experienced progression more frequently compared with those without NF2. Radiographic characteristics of tumors were relatively consistent between groups. While idiopathic SMs remain a relatively benign and highly manageable disease, considering tumor molecular characteristics and broader clinical history is paramount in providing efficacious and individualized patient care.
脊髓脑膜瘤（SM）是一种在美国每年估计有近1000例诊断的疾病，在20%的2型神经纤维瘤病（NF2）患者中出现。这项多机构回顾性队列研究旨在评估1998年至2020年间接受手术的SM患者的临床结局，并按NF2突变状态进行分层。
回顾性审查病历以收集患者人口统计学、临床表现、肿瘤特征、治疗和结局的数据。进行分析以确定全切除（GTR）和肿瘤复发的影像学预测因素，评估NF2相关肿瘤的影像学特征，并确定组间无进展生存期。
研究期间共有166例接受SM手术的患者纳入研究，其中133例为女性（80%）。15例（9%）患者同时诊断为NF2。手术时的平均年龄为58（标准差18）岁。初诊时的平均卡氏功能状态评分为76（标准差11），最常见的初发症状为感觉改变（60%）和无力（59%）。大多数肿瘤位于胸椎（72%）。154例（93%）实现了GTR。8例次全切除患者接受了放射治疗，无一例接受化疗。18例（11%）患者术后出现疾病影像学复发，平均复发时间为4.2年。NF2患者的平均诊断年龄明显更早（33.3[标准差15.4]岁）。NF2患者的进展发生率明显高于其他患者（40%），且时间更短（平均2.8[标准差3.7]年）。NF2患者与非NF2患者之间、接受全切除与次全切除的患者之间以及出现肿瘤复发和未复发的患者之间，包括肿瘤体积、脊髓T2水肿、硬脑膜尾征和钙化在内的影像学特征相似。
在这项对166例接受手术治疗的SM患者的研究中，与无NF2的患者相比，NF2患者就诊更早，进展更早且更频繁。各亚组肿瘤的影像学特征相对一致。虽然特发性SM仍然是一种相对良性且易于管理的疾病，但考虑肿瘤分子特征和更广泛的临床病史对于提供有效且个性化的患者护理至关重要。
