Association of tumor location, extent of resection, and neurofibromatosis status with clinical outcomes for 221 spinal nerve sheath tumors

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Intradural extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas. Given the rarity of this disease and, thus, the limited analyses of clinical outcomes, the authors examined the association of tumor location, extent of resection, and neurofibromatosis (NF) status with clinical outcomes.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>Patients were identified through a search of the University of California, San Francisco, neuropathology database and a separate review of current procedural terminology codes. Data recorded included patient age, patient sex, clinical presentation, presence of NF, tumor type, tumor location, extent of resection (gross-total resection [GTR] or subtotal resection [STR]), and clinical follow-up.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Of 221 tumors in 199 patients (mean age 45 years), 53 were neurofibromas, 163 were schwannomas, and 5 were malignant peripheral nerve sheath tumors. The most common presenting symptom was spinal pain (76%), followed by weakness (36%) and sensory abnormalities (34%). Mean symptom duration was 16 months. In terms of spinal location, neurofibromas were more common in the cervical spine (74% vs 27%, p &lt; 0.001), and schwannomas were more common in the thoracic and lumbosacral spine (73% vs 26%, p &lt; 0.001). Rates of GTR were lower for neurofibromas than schwannomas (51% vs 83%, p &lt; 0.001), regardless of location. Rates of GTR were lower for cervical (54%) than thoracic (90%) and lumbosacral (86%) lesions (p &lt; 0.001). NF was associated with lower rates of GTR among all tumors (43% vs 86%, p &lt; 0.001). The mean follow-up time was 32 months. Recurrence/progression was more common for neurofibromas than schwannomas (17% vs 7%, p = 0.03), although the mean time to recurrence/progression did not differ according to tumor type (45 vs 53 months, p = 0.63). As expected, GTR was associated with lower recurrence rates (4% vs 22%, p &lt; 0.001). According to multivariate analysis, cervical location (OR 0.239, 95% CI 0.110–0.520) and presence of NF (OR 0.166, 95% CI 0.054–0.507) were associated with lower rates of GTR. In a separate model, only GTR (OR 0.141, 95% CI 0.046–0.429) was associated with tumor recurrence.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Resection is an effective treatment for spinal nerve sheath tumors. Neurofibromas were found more commonly in the cervical spine than in other regions of the spine and were associated with higher rates of recurrence and lower rates of GTR than other tumor types, particularly in patients with NF Types 1 or 2. According to multivariate analysis, both cervical location and presence of NF were associated with lower rates of GTR. According to a second multivariate model, the only variable associated with tumor recurrence was extent of resection. Maximal safe resection remains ideal for these lesions; however, patients with cervical tumors or NF should be counseled about their increased risk for recurrence.</jats:p></jats:sec>

Neurofibroma cervicodorsal en una niña con Neurofibromatosis Tipo 1

“…En esta serie, los neurofi bromas representaron el 24% de los tumores siendo su topografía más común la médula cervical (74%). La tasa de resección total fue baja y la recurrencia o progresión elevada (17%) (17) En un estudio multicéntrico realizado por Leonard y cols. entre 1996 y 2006, se identifi caron trece pacientes con compresión de la médula cervical debida a neurofi bromas plexiformes, con edades entre 9 y 61 años; siendo cuatro pacientes menores de dieciocho años.…”

Large Cervical Vagus Nerve Tumor in a Patient with Neurofibromatosis Type 1 Treated with Gross Total Resection: Case Report and Review of the Literature

“…7,8,23,40 Neurofibromas often prove to be the most difficult nerve sheath tumors to achieve GTR. 41 Surgery for resection of a vagus tumor mass should be a multiple disciplinary endeavor. 15 In our case, both otolaryngologists and neurosurgeons were employed to treat this tumor in a complicated anatomical area.…”

Intradural Extramedullary Spinal Neoplasms: Radiologic-Pathologic Correlation

“…Spinal neurofibromas are most common in the cervical region, with higher recurrence rates and lower rates of gross total resection compared with other tumor types (48). Cranial nerves are virtually never involved (46).…”