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      Non-Syndromic Spinal Schwannomas: A Novel Classification

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          Abstract

          Schwannomas are the most frequent primary tumors of the spine with an incidence of 0.3–0.5/100,000 person per year. Current treatment for non-syndromic spinal schwannomas is total resection of the tumor with preservation of neurovascular structures. This study aims to report neurologic and radiologic outcome following treatment of non-syndromic spinal schwannomas along with a novel tumor classification used in our clinic. A retrospective case series was carried out with a patient sample of 82 male and female patients with non-syndromic spinal schwannomas. All patient data were retrospectively collected from the hospital records. As a routine procedure, after admittance and primary evaluation, patients’ tumors were classified using CT or MRI in accordance with our proposed classification method, which employs a dual designation method with tree groups (A, B, and C) for tumor volume and four types (I, II, III, and IV) for tumor localization. Subsequent resection surgery was followed by neurological assessments and follow up at 45th, 180th, and 360th postoperative day. Along with Karnofsky performance status scale, pain, sensory deficits, and motor weakness were scored to assess neurologic recovery. Our finding indicates that patients with different tumor types significantly differ in their neurological scores and show consistent but differential neurological recovery at early and late time points postsurgery. Complications during and postsurgery were minimal, occurring only in two patients. Our findings further reinforce the established safety of total resection operations and indicate that our proposed classification is a simple, effective tool that has proven helpful in preoperative planning and avoiding unnecessary surgical approaches.

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          Most cited references33

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          Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases.

          Are spinal schwannomas as benign as we think? To what extent do patients recover? Are patients prone to develop late complications such as cystic myelopathy or symptomatic spinal deformity? Is their life expectancy compromised? In an effort to answer these questions, the authors analyzed the long-term outcome for 187 patients from one neurosurgical department with surgically treated spinal schwannoma. Median follow-up period was 12.9 years (2454 patient years). One-fifth of the patients considered themselves free of symptoms at follow-up examination. The most common late complaint was local pain (46%), followed by radiating pain (43%), paraparesis (31%), radicular deficit (28%), sensory deficit due to a spinal cord lesion (27%), and difficulty voiding (19%). Late complications occurred in 21% of the patient population, including cystic myelopathy (2%), spinal arachnoiditis (6%), spinal deformity (6%), and troublesome pain (7%). Life expectancy of the patients corresponded to that of the general population.
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            Diagnostic criteria for schwannomatosis.

            The neurofibromatoses are a diverse group of genetic conditions that share a predisposition to the development of tumors of the nerve sheath. Schwannomatosis is a recently recognized third major form of neurofibromatosis (NF) that causes multiple schwannomas without vestibular tumors diagnostic of NF2. Patients with schwannomatosis represent 2.4 to 5% of all patients requiring schwannoma resection and approximately one third of patients with schwannomatosis have anatomically localized disease with tumors limited to a single limb or segment of spine. Epidemiologic studies suggest that schwannomatosis is as common as NF2, but that familial occurrence is inexplicably rare. Patients with schwannomatosis overwhelmingly present with pain, and pain remains the primary clinical problem and indication for surgery. Diagnostic criteria for schwannomatosis are needed for both clinicians and researchers, but final diagnostic certainly will await the identification of the schwannomatosis locus itself.
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              Giant invasive spinal schwannomas: definition and surgical management.

              Confusion exists regarding the term giant spinal schwannoma. There are a variety of nerve sheath tumors that, because of their size and extent, justify the label "giant schwannoma." The authors propose a classification system for spinal schwannomas as a means to define these giant lesions. The classification is confined to tumors that are essentially intraspinal, with or without extraspinal components. Lesions that erode the vertebral bodies (VBs) and extend posteriorly and laterally into the myofascial planes are classified as giant "invasive" spinal schwannomas. The records of patients with giant invasive spinal schwannoma were analyzed. The radiological features, operative approaches, and intraoperative findings were noted. Ten patients with giant invasive tumors were surgically treated over the last 8 years. Six patients were male. Erosion of the posterior surface of the VBs was the diagnostic finding demonstrated on plain x-ray films. Magnetic resonance imaging delineated the extent of the tumors and helped in preoperative planning. Radical excision of the tumors in multiple stages was possible in eight of the 10 patients. Dural reconstruction was required in four patients. All patients required fusion, and an additional stabilization procedure was undertaken in three patients. The authors conclude that giant invasive schwannomas are uncommon lesions and propose a new classification system. Because of their locally "invasive" nature and extension in all directions, careful preoperative planning of the surgical approach is very important. Although radical excision is possible and promises good results, recurrences may occur and multiple surgical procedures may be required.
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                Author and article information

                Contributors
                Journal
                Front Neurol
                Front Neurol
                Front. Neurol.
                Frontiers in Neurology
                Frontiers Media S.A.
                1664-2295
                17 July 2017
                2017
                : 8
                : 318
                Affiliations
                [1] 1Department of Neurosurgery, Acıbadem University School of Medicine , Istanbul, Turkey
                Author notes

                Edited by: Gordon Li, Stanford University, United States

                Reviewed by: Brigitta G. Baumert, Maastricht University Medical Centre, Netherlands; Jonathon Parkinson, University of Sydney, Australia

                *Correspondence: Ibrahim Sun, halilibrahim.sun@ 123456acibadem.edu.tr

                Specialty section: This article was submitted to Neuro-Oncology and Neurosurgical Oncology, a section of the journal Frontiers in Neurology

                Article
                10.3389/fneur.2017.00318
                5511849
                28769861
                d85c2400-8a81-450d-b20f-de7e2e5d991a
                Copyright © 2017 Sun and Pamir.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 28 March 2017
                : 19 June 2017
                Page count
                Figures: 7, Tables: 4, Equations: 1, References: 35, Pages: 9, Words: 4975
                Categories
                Neuroscience
                Original Research

                Neurology
                spinal schwannoma,resection surgery,tumor classification,postoperative recovery,spinal tumor

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