Sporadic spinal schwannomas and neurofibromas - a review (original) (raw)
Related papers
Primary Spinal Schwannoma: A Single Center Study Of 37 consecutively Operated Cases
Nepal journal of neuroscience, 2022
Background: Primary spinal Schwannoma (PSS) remains uncommon nerve sheath tumor of the spine with male preponderance and presenting in fourth to fifth decades. They arise from the Schwann cells in sensory root as PSS or part of Neurofibromatosis 2. Methods: This is a retrospective study of histopathologically confirmed cases of PSS treated between 2010 to 2021. They were operated and age, sex, clinic-radiological findings, surgical technique used and outcome studied. Laminectomy or laminoplasty or combined anterior-posterior approach was used with either an intracapsular or extracapsular tumor removal. Follow-up was at 15 days, 2 months, 6 months and yearly intervals. Results: There were 37 cases operated with age ranging from 16 to 81 years and majority in the 21-40-year group (40%) followed by 41 to 60 years (32%). There were 22 males and 15 females. Localized pain in the back or cervical region was the commonest finding (75%) followed by myelopathy in 38%, radiculopathy in 35% and bladder disturbances in 13%. They were most common in the lumbar region (35%) followed by thoracic (30%). With relation to the level and sex, the cervical level showed equal sex distribution while the rest had male preponderance Conclusions: PSS although can frequently present to the spine surgeon and the diagnosis is clinched with help of an MRI. Surgery remains the main modality of treatment either by laminectomy, laminoplasty or minimal invasive spine surgery. The nerve root of origin must be preserved in all that can be aided with help of intraoperative neurophysiological monitoring.
Spinal schwannomatosis: case report of a rare condition
Turkish neurosurgery, 2008
Schwannomatosis is a rare tumour syndrome characterized by more than one schwannoma without any sign of neurofibromatosis (NF). A 22-year-old male patient was admitted with weakness in his extremities. Neurological examination revealed a tetraparesis syndrome below the C6 level. Magnetic resonance (MR) imaging showed a demarcated mass with strong enhancement at the C4-7 levels. The patient improved rapidly after removal of the tumour. The histological diagnosis was schwannoma. Two years later, the same patient was admitted with the complaint of severe low back and leg pains. The neurological examination was normal except bilateral positive straight leg raising test and dysesthesia at the L3,4 and 5 dermatomes. MR showed a wellcircumscribed lesion at L4-5. Laminectomy and tumour excision relieved his complaints. The histological diagnosis was schwannoma. A detailed clinical examination and MR scanning of the central nervous syndrome excluded NF2. In conclusion, although schwannomatos...
Neurology Research International, 2017
Background.Spinal schwannomas are common benign spinal tumors. Their treatment has significantly evolved over the years, and preserving neurological functions has become one of the main treatment goals together with tumor resection.Study Design and Aims.Retrospective review focused on clinical assessment, treatment techniques, and outcomes.Methods.A retrospective study on our surgical series was performed. Clinical and operative data were analyzed. In regard to neurophysiologic monitoring, patients were retrospectively divided into two groups comparing the outcomes before and after introduction of routine intraoperative neurophysiology tests.Results.From 1951 to 2010, 367 patients overall were treated. Diagnosis was obtained using angiography and/or myelography (pre-CT era), MRI, or CT scan. A posterior spinal approach was used for most patients; complex approaches were adopted for treatment of giant/dumbbell tumors. A trend of neurophysiology monitoring decreasing the rate of post-...
Spinal Schwannoma : Scrutinizing the Armamentarium of a Single Institute
Journal of the Royal Medical Services, 2018
Objectives: To investigate the clinical characteristics, epidemiological features of spinal schwannomas diagnosed at a single institution. Methods: A retrospective study carried out of all consecutives 67-patients presented with spinal schwannoma treated at the neurosurgery department in King Hussein Medical Center. Clinical information, imaging studies, and biopsy results were compiled from all consecutive patients (all cases were included) treated in our setup over a period of 10-years from January 2006 to January 2016. Results: Study cohort consisted of 38-females (56.71%) and 29-males (43.28%), with ages ranging from 17 to 75 years (mean: 41.45 ± 13.74 yr). The most common initial symptom was radicular pain (46 patients, 68.65%). The most common regions of involvement were the lumbar spine (37 patients, 55.22%), followed by cervical (16 patients, 23.88%), thoracic segments (12 patients, 17.91%) and sacral region (2 patients, 2.98%). In the total population of 67-cases, the schwannomas were intradural extramedullary in 65-cases (97.01%) and in 2-cases it was extradural (2.98%). Three cases (4.47%) recurred locally. Conclusion: Spinal schwannomas are benign tumors that can cause significant neurological deficits. Lumbar spine is the most common site of spinal schwannomas according to this study. Surgery for spinal schwannomas usually results in excellent postoperative clinical outcomes. Schwannoma is generally separable from the underlying nerve. In cases of subtotal removal, there could be a recurrence.
Romanian Neurosurgery
Schwannomatosis is a rare tumor syndrome characterized by the presence of multiple benign non-vestibular, non-intradermal schwannomas and by the absence of neurofibromatosis type 1 or 2 syndromes. Multiple schwannomas are clinically and genetically distinct from neurofibromatosis, and the main treatment in case of symptomatic lesions is represented by surgical resection. In case of asymptomatic lesions, the indicated treatment is neuroimaging follow-up (MRI). We are presenting the case of a patient with three schwannomas of the cauda equina, as well as the treatment strategy in the case of this rare pathology.
Management of Spinal Schwannomas in Gabriel Touré Hospital: Review of 11 Cases
Surgical Science
Background: Spinal schwannomas are common tumors of spinal neoplasm and account for about 25% of intradural spinal cord tumors in adults. They are generally benign and slow-growing. Advanced in radiologic and surgical techniques have brought about better surgical results. The goal of surgical treatment must be total resection if possible. In this report, the authors present the incidence, clinical presentation, localization, and results of surgically treated spinal schwannomas. The results of a literature review are also presented. Methods: Eleven consecutive patients with histologically confirmed spinal schwannomas were treated from January 2013 to December 2016 in the neurosurgical department of Gabriel Touré Hospital, Bamako, Mali. Neuroradiological diagnosis was made, CT scan in 7 patients, and MRI in 4 patients. All patients were operated on via the posterior approach. All cases were surgically excised, and they were confirmed to be schwannomas by pathologists. The patients were followed for 6 to 38 months (mean 28 months). Functional outcome was assessed using the motor grade and sensory change. Results: There were 11 patients with 7 (63.6%) males and 4 (36.4%) females. The mean age was 40.3 years (range 23-62 years). The most common symptom at the time of diagnosis was radicular pain in 9 (81.8%) patients followed by motor weakness in 8 (72.7%) patients. The most frequent site of spinal schwannomas was the thoracic region in 5 (45.5%) patients. During surgery, Gross-total resection was achieved in 8 patients (72.7%) and subtotal removal in 3 (27.3%) patients. Histological findings were benign schwannoma in all How to cite this paper:
Turkish Neurosurgery, 2015
tumors, particularly in adults (8). Rarely, SS have possibility of malignancy and therefore the recommended primary treatment of choice is gross-total resection (GTR) with preservation of neurological function. GTR of these lesions, which is the main goal of surgical treatment, leads to good results. Giant dumbbell SS, which extends anteriorly to invade the bones, vascular structures, and soft tissues and fill the abdominal and thoracic cavities as same as extradural location █ InTRODuCTIOn S pinal schwannomas (SS) are well known, macroscopically solid encapsulated, spindle cell mesenchymal tumors which are called neurinomas in the literature. Although they are mostly benign entities derived from neoplastic schwann cells, SS have possibility of malignant transformation (10,15). SS represent the most common intradural extramedullary lesions, accounting for about one fourth of all nerve sheath AIM: Spinal schwannomas (SS) represent the most common intradural extramedullary lesions, accounting for approximately 24% of all nerve sheath tumors in adults. Schwannomas have infrequent, but existent possibility of malignancy. In this study, long-term outcomes of 49 consecutive SS have been presented. MATERIAL and METhODS: Medical records were retrospectively reviewed in 371 cases of spinal tumors who underwent surgery between the years 2005 and 2014. Cases confirmed as schwannoma histopathologically were included in this study. Patients' complaints, localizations, recurrence rate and complications were evaluated. RESuLTS: Forty-nine cases were detected in 47 (26 female, 21 male) patients. The mean age was 45.8±13.7 years. The mean follow-up period was 61.4±21.5 months. The most common complaint was local pain. Eleven were cervical, ten thoracic, twentyfour lumbar, and four in the sacral spine. Thirty-three cases were intradural-extramedullary, fifteen cases were the extradural type, and one case was the extra-intradural type. Recurrence rate was 4.08%. Gross-total resection (GTR) was achieved in forty-seven patients. The most common complications were surgical site infection and intraoperative instability that were seen in three patients each. Posterior instrumentation was performed in two patients. COnCLuSIOn: SS is mostly benign and intradural-extramedullary. To treat patients with SS, there is no need for adjuvant treatments; GTR with preservation of neurological functions is the best treatment to relieve patients' complaints and to reduce the recurrence rate of SS. To avoid serious complications, we recommend intraoperative neurophysiological monitoring and laminoplasty, especially in young patients. Dumbbell SS may require extensive bone resection. Posterior instrumentation can be used if instability occurs.
Two sporadic spinal neurofibromatosis patients with malignant peripheral nerve sheath tumour
European Journal of Medical Genetics, 2009
We analyzed two unrelated male patients in whom neurofibromatosis type 1 (NF1) was not suspected until they presented with malignant peripheral nerve sheath tumours (MPNSTs) in their thirties and forties, respectively. Patient A presented with progressive peroneus paresis due to a rapidly growing MPNST in the thigh. MRI examination revealed multiple symmetrical spinal neurofibromas in this patient as well as in patient B who presented at the age of 42 with paraparesis and an MPNST at spinal level L4. Dermal features in both patients were strikingly mild, therefore both patients were considered belonging to the NF1-subform of spinal neurofibromatosis (SNF). The novel NF1 mutations identified, i.e. splice mutation, c.7675þ1G > A, in patient A and two alterations, p.Cys1016Arg and p.2711delVal, located in trans in patient B support the notion that the phenotype of SNF may be related to mutations with possible residual functionality. The MPNSTs of both patients showed LOH affecting chromosome 17 including the NF1 locus. Furthermore, a truncating TP53 mutation was identified in the tumour of patient A. Both alterations are frequent findings in NF1-associated MPNSTs. To our knowledge these are the first MPNST patients with the clinical phenotype of SNF. The clinical course observed in these two patients suggests that nodular plexiform neurofibromas and spinal-nerve-root neurofibromas which may be asymptomatic for a long time and, hence, unrecognized in SNF patients bear the risk for malignant transformation.
Single root multiple spinal schwannomas: Case report, treatment strategy and review of literature
International Journal of Surgery Case Reports
INTRODUCTION: Schwannomatosis is defined as multiple schwannomas without presence of neurofibromatosis and is a rare pathology. In vast majority of cases the schwannomas grow from different nerve roots or peripheral nerves. PRESENTATION OF CASE: A 52-year-old woman presented with multiple intradural schwannomas arranged in a chain along the spinal canal causing significant compression. The lesions were successfully removed using a left side en-bloc hemilaminectomy technique in order to preserve maximal stability of the posterior column. Back and leg pain resolved completely. Tendon reflexes returned to normal shortly. There was decreased pain sensation in the distribution of the left L3 spinal root. DISCUSSION: The traditional surgical strategy for posterior approach by laminectomy or laminotomy is sometimes complicated with instability or deformation of the vertebral column that requires surgical stabilization. We performed a one side en-bloc hemilaminectomy thus maintaining the integrity of the muscles and ligaments on the opposite side and preserving maximal stability of the vertebral column. Densely adherent tumors required careful sharp dissection and separation under neurosurgical monitoring and stimulation for recognition and preservation of spinal roots. An additional tumor was discovered by exploration of the spinal canal using an endoscope. CONCLUSION: Multiple spinal cord schwannomas that are growing along the same part of the vertebral column can be safely removed by one-sided hemilaminectomy with preservation of the integrity of the muscles and ligaments on the opposite side and thus maintain spinal stability. The 30 • endoscope can be a good tool for visual exploration of the spinal canal.