Presenting characteristics and clinical outcomes of idiopathic versus neurofibromatosis type 2-associated spinal meningiomas: a retrospective institutional experience.

Abstract

Objective: 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.

Methods: 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.

Results: 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.

Conclusions: 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.