Spinal Schwannomas Research Articles

Long-Term Functional Outcomes Following Surgical Treatment of Spinal Schwannomas: A Population-Based Cohort Study

Spinal schwannomas are the second most common form of primary intradural spinal tumor. Despite being benign, they may cause spinal cord compression and subsequently acute or chronic neurological dysfunction. The primary treatment is surgical resection. The aim of this study was to identify pre- and postoperative predictors of favorable outcomes after surgical treatment for spinal schwannoma. All adult patients surgically treated for spinal schwannoma between 2006 and 2020 were eligible for inclusion. Medical records and imaging data were retrospectively reviewed. The primary outcome measures were neurological improvement according to the modified McCormick Scale (mMC) and changes in motor deficit, sensory deficit, gait disturbance, bladder dysfunction, and pain at long-term follow-up. In total, 180 patients with a median follow-up time of 4.4 years were included. Pain was the most common presenting symptom (87%). The median time between symptom presentation and surgery was 12 months, while the median time between diagnosis (first MRI) and surgery was 3 months. Gross total resection (GTR) was achieved in 150 (83%) patients and the nerve root could be preserved in 133 (74%) patients. A postoperative complication occurred in 10 patients (5.6%). There were significant postoperative improvements in terms of motor, sensory, gait, and bladder functions, as well as pain (p < 0.001). Of these symptoms, bladder dysfunction was the one most often improved, with complete symptom resolution in all cases. However, no other predictors of improvement could be identified. There were three cases of recurrence after GTR and nine cases of regrowth after STR. Reoperation was performed in six (3.3%) cases. GTR was associated with a significant improvement in neurological status at long-term follow-up and increased the chance of progression-free survival.

Long-term postoperative outcomes of spinal cellular schwannoma: study of 93 consecutive cases

BACKGROUND CONTEXTCellular schwannoma (CS) is a rare tumor that accounts for 2.8%–5.2% of all benign schwannomas. There is a dearth of up-to-date information on spinal CS in the literature. PURPOSEThe aims of this study were to identify the proportion of CS cases amongst spinal benign schwannoma, describe the clinical features of spinal CS, and identify prognostic factors for local recurrence by analyzing data from 93 consecutive CS cases. STUDY DESIGNRetrospective review. PATIENT SAMPLEWe analyzed 93 PSGCT screened from 1,706 patients with spine CS who were treated at our institute between 2008 and 2021. OUTCOME MEASURESDemographic, radiographic, operative and postoperative data were recorded and analyzed. METHODSWe compared the clinical features of spinal CS from the cervical, thoracic, lumbar and sacral segments. Prognostic factors for local recurrence-free survival (RFS) were identified by the Kaplan-Meier method. Factors with p≤.05 in univariate analysis were subjected to multivariate analysis by Cox regression analysis. RESULTSThe proportion of spinal CS in all benign schwannomas was 6.7%. The mean and median follow-up times for the 93 patients in this study were 92.2 and 91.0 months respectively (range 36–182 months). Local recurrence was detected in 11 cases, giving an overall recurrence rate of 11.7%, with one patient death. Statistical analysis revealed that tumor size ≥5 cm, intralesional resection, and Ki-67 ≥5% were independent negative prognostic factors for RFS in spinal CS. CONCLUSIONSWhenever possible, en bloc resection is recommended for spinal CS. Long-term follow-up should be carried out for patients with tumor size ≥5 cm and postoperative pathological Ki-67 ≥5%.

Development of a novel prediction model for differential diagnosis between spinal myxopapillary ependymoma and schwannoma

Spinal myxopapillary ependymoma (MPE) and schwannoma represent clinically distinct intradural extramedullary tumors, albeit with shared and overlapping magnetic resonance imaging (MRI) characteristics. We aimed to identify significant MRI features that can differentiate between MPE and schwannoma and develop a novel prediction model using these features. In this study, 77 patients with MPE (n = 24) or schwannoma (n = 53) who underwent preoperative MRI and surgical removal between January 2012 and December 2022 were included. MRI features, including intratumoral T2 dark signals, subarachnoid hemorrhage (SAH), leptomeningeal seeding, and enhancement patterns, were analyzed. Logistic regression analysis was conducted to distinguish between MPE and schwannomas based on MRI parameters, and a prediction model was developed using significant MRI parameters. The model was validated internally using a stratified tenfold cross-validation. The area under the curve (AUC) was calculated based on the receiver operating characteristic curve analysis. MPEs had a significantly larger mean size (p = 0.0035), higher frequency of intratumoral T2 dark signals (p = 0.0021), associated SAH (p = 0.0377), and leptomeningeal seeding (p = 0.0377). Focal and diffuse heterogeneous enhancement patterns were significantly more common in MPEs (p = 0.0049 and 0.0038, respectively). Multivariable analyses showed that intratumoral T2 dark signal (p = 0.0439) and focal (p = 0.0029) and diffuse enhancement patterns (p = 0.0398) were independent factors. The prediction model showed an AUC of 0.9204 (95% CI 0.8532–0.9876) and the average AUC for internal validation was 0.9210 (95% CI 0.9160–0.9270). MRI provides useful data for differentiating spinal MPEs from schwannomas. The prediction model developed based on the MRI features demonstrated excellent discriminatory performance.

Giant Invasive Schwannomas of C1 Root – Technical Challenges and Report of the First Two Cases in Literature

Abstract Giant invasive schwannomas (GISs) have been defined as different from other spinal schwannomas. C1 schwannomas are rare and often described with other high cervical tumors. GISs of C1 have not been previously described. Two cases of GIS are reported. In both patients, presenting with myelopathy, the lesions were extradural and intradural with posterior, lateral, and anterior extensions, compressing the cervicomedullary junction (CMJ) and displacing it. The V3-4 segments of the vertebral artery (VA) were closely associated. A posterolateral approach was used to initially expose and debulk the extradural portion of the lesion, which allowed access to the intradural component. The latter was followed anterior and/or posterior to the CMJ and excised completely. The tumors were dissected at the VA. Bleeding from the perivertebral venous plexus was controlled using topical hemostats. A small bit of tumor was left in a blind area anterior to the C1 arch in one patient. There were no complications and both patients improved in the postoperative period. GIS of the C1 are rare lesions in a difficult location. Complex relationships to the CMJ, lower cranial nerves, VA, and bony spine need a careful preoperative understanding of the anatomy and planning of surgery.

Radiographic Features of Spinal Meningioma and Schwannoma: A Novel Specific Feature—Ginkgo Leaf Sign

Radiographic Features of Spinal Meningioma and Schwannoma: A Novel Specific Feature—Ginkgo Leaf Sign

Multiple Cranial Nerve Involvement in a Complex Case of MISME Syndrome in a Paediatric Patient: A Case Report

Neurofibromatosis 2 (NF2) is characterised by numerous tumours in the central and peripheral nervous systems due to NF2 gene abnormalities that cause the tumour suppressor protein, Merlin, to disappear. Often referred to as Multiple Inherited Schwannomas, Meningiomas, and Ependymomas (MISME), a distinctive characteristic of NF2 is bilateral vestibular schwannomas manifesting in late adolescence with symptoms such as sensorineural hearing loss, tinnitus, and balance issues. Two distinct phenotypes, Wishart and Feiling-Gardner, characterise NF2. This case report discusses the case of a paediatric patient who presented with bilateral hearing loss, giddiness, and blurring of vision and sought a Magnetic Resonance Imaging (MRI) examination which revealed bilateral vestibular schwannomas, non vestibular schwannomas, left sphenoid wing meningiomas, multidirectional spinal schwannomas, spinal nerve sheath tumours, and lesions in the retroperitoneal region. Despite an absent family history, significant involvement of cranial nerves strongly indicates classical NF2. Management focuses on preserving function, and surgery is contemplated for symptomatic lesions and tumours causing cord compression. Gamma Knife radiosurgery and targeted therapies have been investigated.

Spinal schwannoma classification based on the presumed origin with preoperative MR images

Spinal schwannoma classification based on the presumed origin with preoperative MR images

Unusual Anterior Neck Swelling: Cervical Spinal Cord Schwannoma

AbstractSchwannomas are typically solitary, well-encapsulated, benign tumors running along or attached to a nerve. An intradural-extramedullary cervical spinal schwannoma, which first manifests as a swelling in the anterior neck, has not been reported to the best of our knowledge. We present the case of a 69-year-old patient complaining of a gradually worsening painful left cervical swelling for over 8 years. First, a posterior spinal midline approach was performed for the resection of the tumor and the tumor portion in the vertebral canal was totally removed. The second operation, the anterolateral approach, was planned to be executed in a second surgical session. The patient was discharged from the hospital without neurological deficits. Histopathological diagnosis was a schwannoma. The first aim of surgery is to treat neurological deficits in patients with cervical intraspinal schwannomas with/without extension into the extra-vertebral paravertebral neck regions. The surgical strategy combines the posterior midline and the anterolateral cervical approaches in the same session or at different times.

NIMG-07. LONG-TERM FOLLOW-UP OF SCHWANNOMA GROWTH BEHAVIOR IN ADULT SCHWANNOMATOSIS PATIENTS USING WHOLE-BODY MRI

Abstract BACKGROUND The schwannomatoses include NF2-related schwannomatosis (NF2-SWN, formerly known as “NF2”) and non-NF2-related schwannomatosis (e.g. SMARCB1- and LZTR1-related schwannomatosis) and are caused by distinct germline pathogenic variants on chromosome 22. Patients may develop cranial, peripheral, and/or spinal nerve schwannomas. Except for vestibular schwannomas, the long-term growth behavior of schwannomas is unknown but this is important to guide patient surveillance and selection for treatment. Whole-body MRI (WBMRI) can detect whole-body schwannoma burden. METHODS 16 NF2-SWN and 12 non-NF2-SWN patients with WBMRI acquired between 2007-2010 underwent a repeat WBMRI between 2018-2022. Non-vestibular schwannomas were segmented on short tau inversion recovery (STIR) sequences. Tumor volume was calculated using a 3D tumor quantification software (3DQI). Tumor growth and shrinkage were defined as a volume change ³20% over the entire study period. RESULTS Median time between scans was 10.3 years. Median age was 43.5 years at baseline and 54 years at follow-up. Excluding resected tumors, 102 schwannomas (49 NF2-SWN, 53 non-NF2-SWN) were analyzed. 62/102 (61%) tumors grew >20% (65% NF2-SWN, 57% non-NF2-SWN); median growth was 96% in NF2-SWN and 94% in non-NF2-SWN. 24/102 (24%) tumors shrank >20% (20% NF2-SWN, 26% non-NF2-SWN); median shrinkage was 49% in NF2-SWN and 40% in non-NF2-SWN. 29 new tumors developed between scans (16 NF2-SWN, 13 non-NF2-SWN). Of 32 NF2-SWN tumors exposed to systemic or radiation therapy, 63% grew and 31% shrank. Of 7 non-NF2-SWN tumors exposed to treatment, 29% grew and 42% shrank. 11 non-NF2-SWN tumors shrank spontaneously without treatment. Spontaneous tumor shrinkage was not observed in NF2-SWN. CONCLUSIONS About 60% of SWN-associated schwannomas grow significantly over a decade. Spontaneous shrinkage is only observed in non-NF2-SWN tumors whereas, in NF2-SWN patients, about 60% of tumors grow despite treatment, suggesting a more aggressive tumor phenotype in NF2-SWN. Correlation of MRI findings with patient-reported outcomes and hormone exposure history are ongoing.

INNV-04. RAPID CLINICAL AND RADIOLOGICAL RESPONSE TO BEVACIZUMAB IN A PATIENT WITH SCHWANNOMATOSIS

Abstract Limited evidence exists for optimal management of schwannomatosis, the least common neurofibromatosis syndrome characterized by multiple schwannomas and intractable pain in the absence of vestibular schwannomas and germline NF2 mutation. We present a case of a 32-year-old man with a family history of schwannomas who had been dealing with multiple paraspinal schwannomas throughout entire spine, neurological deficits and chronic severe pain since high school. He had multiple laminectomies with resection of schwannomas. By the time neurooncologist saw the patient he had been on daily narcotics for more than 10 years, was not ambulatory due to paraparesis and was dependent on chronic Foley catheter for urination. He was found to have SMARCB1 c.93G >A germline mutation that has not been reported before. Total spine MRI demonstrated further progression of multiple spinal schwannomas with cord compression. The patient started on bevacizumab 10 mg/kg IV every 4 weeks (the first dose 5 mg/kg) but had to have an 8-week interval between his second and third cycle because of slowly healing pressure ulcers over bilateral upper buttocks. The patient started to show improvement in leg movements and strength after 2 cycles, his urinary catheter was discontinued, and he started urinating on his own after 3 cycles of bevacizumab. MRI obtained after 3 cycles demonstrated decreasing size and enhancement of multiple schwannomas. The patient continued bevacizumab every 4 – 6 weeks and continued with clinical improvement of strength and mobility. He started to stand with support for several minutes after 6 cycles of bevacizumab and reported better control of pain. He started outpatient physical therapy sessions. The plan is to continue bevacizumab therapy long term. This case demonstrated efficacy of bevacizumab as an alternative treatment option to improve quality of life and avoid surgeries in patients with this rare debilitating condition.

Upper cervical intramedullary schwannoma of the spinal cord presenting with myelopathy: illustrative case.

Intramedullary schwannomas account for 1.1% of all spinal schwannomas. Preoperative diagnosis is best accomplished by thoroughly evaluating clinical and radiological characteristics, accompanied by a high index of suspicion. The authors report a case of C2-3 intramedullary schwannoma in a young male who presented with neck pain and vertigo. The current literature is also reviewed. The authors reviewed the data of a young male with a 2-month history of neck pain and vertigo. Magnetic resonance imaging of the brain and cervical spine showed an intramedullary mass at C2-3 with a syrinx extending into the cervicomedullary junction. Laminectomy, myelotomy, and microsurgical excision of the mass under intraoperative neurological monitoring (IONM) were done. Postoperative pathology reported the specimen as a schwannoma. Gross-total resection of a schwannoma using IONM is the treatment of choice because of the lesion's benign nature, a better prognosis, and defined cleavage plane. Schwannomas should be included in the differential diagnosis of intramedullary spinal tumors. Because of its progressive nature, early surgery is recommended in symptomatic patients.

Spinal Schwannomas; Classification, Management And Outcomes.

Schwannomas are benign tumours of the peripheral nerve sheath. When they occur in spine, they are most commonly found in intradural-extramedullary location. Surgery is the mainstay of treatment. Radiation has a limited role in the management of residual or recurrent lesions not suitable for surgery. Here we discuss the existing literature on the outcomes of spinal schwannoma after surgery.

P128. Surgical resection of spinal schwannomas: clinical features and neurological outcomes

P128. Surgical resection of spinal schwannomas: clinical features and neurological outcomes

Automated Detection and Diagnosis of Spinal Schwannomas and Meningiomas Using Deep Learning and Magnetic Resonance Imaging.

Spinal cord tumors are infrequently identified spinal diseases that are often difficult to diagnose even with magnetic resonance imaging (MRI) findings. To minimize the probability of overlooking these tumors and improve diagnostic accuracy, an automatic diagnostic system is needed. We aimed to develop an automated system for detecting and diagnosing spinal schwannomas and meningiomas based on deep learning using You Only Look Once (YOLO) version 4 and MRI. In this retrospective diagnostic accuracy study, the data of 50 patients with spinal schwannomas, 45 patients with meningiomas, and 100 control cases were reviewed, respectively. Sagittal T1-weighted (T1W) and T2-weighted (T2W) images were used for object detection, classification, training, and validation. The object detection and diagnosis system was developed using YOLO version 4. The accuracies of the proposed object detections based on T1W, T2W, and T1W + T2W images were 84.8%, 90.3%, and 93.8%, respectively. The accuracies of the object detection for two spine surgeons were 88.9% and 90.1%, respectively. The accuracies of the proposed diagnoses based on T1W, T2W, and T1W + T2W images were 76.4%, 83.3%, and 84.1%, respectively. The accuracies of the diagnosis for two spine surgeons were 77.4% and 76.1%, respectively. We demonstrated an accurate, automated detection and diagnosis of spinal schwannomas and meningiomas using the developed deep learning-based method based on MRI. This system could be valuable in supporting radiological diagnosis of spinal schwannomas and meningioma, with a potential of reducing the radiologist's overall workload.

Factors Related to the Deterioration of Postoperative Lower Back Pain in Hemilaminectomy Approach for Lumbar Spinal Schwannoma Resection.

This study aimed to explore the related risk factors in patients who underwent hemilaminectomy for lumbar spinal schwannoma resection and who experienced deterioration of postoperative lower back pain in comparison to preoperative pain levels. This retrospective study recruited 61 patients from the First Affiliated Hospital of An Hui Medical University between January 2018 and June 2019. All data were collected from clinical records and analyzed at 1-month and at 1-year follow-up. The visual analog scale (VAS) was used to evaluate pain, and neurologic function was assessed using the Modified McCormick Scale. Intraoperative neurophysiological monitoring was used to assess neuronal integrity and mitigate injury. Statistical analysis of the data was performed using the SPSS version 19 software. Preoperative pain improved dramatically in the 1-year follow-up (VAS: preoperative, 3.84±2.19; 1-year follow-up, 2.13±2.26; P<0.001). The pain-improved group and worsened group showed a significant difference at 1-month (VAS: 1.76±1.56; 5.54±1.26; P<0.05) and at 1-year (VAS: 0.83±1.09; 4.80±1.58; P<0.05) follow-up. The pain-improved and worsened groups had a significant difference in tumor size and hemilaminectomy removal segments at 1-month and 1-year follow-up, but A-train occurrence on electromyography could only be seen as a statistical difference in the 1-month follow-up. Logistic regression analysis revealed that tumor size was an independent risk factor for postoperative lower back pain deterioration. The hemilaminectomy approach is a safe and effective method that can dramatically relieve pain in spinal lumbar schwannoma resection. Tumor size is an independent risk factor for postoperative lower back pain. A-train on spontaneous electromyography has been shown to be a reliable predictive factor for the evaluation of postoperative lower back pain. However, further detailed analysis of A-train characteristics can provide a more accurate warning during surgery.

Is laminectomy necessary for C1-C2 epidural schwannomas?

Spinal schwannomas often require laminectomy for gross total resection. However, laminectomy may not be necessary due to the unique anatomy of epidural schwannomas at the C1-2 level, even with the intradural part. This study aimed to determine the need for laminectomy by comparing factors between patients who underwent laminectomy and those who did not and to identify the benefits of not performing laminectomy. Fifty patients with spinal epidural schwannoma confined to C1-C2 level were retrospectively collected and divided into groups based on whether laminectomy was intended and performed. In all cases where laminectomy was conducted, patients underwent laminoplasty using microplate-and-screws, which deviates from the conventional laminectomy approach. Tumor characteristics were compared, and a cut-off value for laminectomy was determined. Outcomes were compared between groups, and factors influencing laminectomy were identified. Postoperative changes in cervical curves were measured. The diameter of the intradural part of the tumor was significantly longer in the laminectomy performed group, with a 14.86mm cut-off diameter requiring laminectomy. Recurrence rates did not differ significantly between groups. Surgery time was substantially longer for the laminectomy performed group. No significant changes were observed in Cobb's angles of Oc-C2, C1-C2, and Oc-C1 before and after surgery. The study showed that the diameter of the intradural part of the tumor influenced the decision to perform laminectomy for removing epidural schwannomas at C1-C2. The cut-off value of the diameter of the intradural part of the tumor for the laminectomy was 14.86mm. Not performing laminectomy can be a viable option with no significant differences in removal and complication rates.

Spinal Intramedullary Schwannoma of the Conus

AbstractSchwannomas are one of the most common primary spinal tumors representing 30% of all intraspinal lesions. Intramedullary schwannomas constitutes 0.3% of all intraspinal tumors and approximately 1% of spinal cord schwannomas. Majority of the tumors are seen in the cervical (58%), followed by thoracic (32%) and lumbar (10%) regions. Very few are reported at the level of conus medullaris. The important radiologic features of intramedullary schwannoma are predominant extramedullary component, intramedullary spinal tumor with a thickened and enhancing spinal nerve root, absent syrinx, enhancing well with contrast, and sharp margins. The aim of surgery in intramedullary spinal schwannomas is total removal whenever possible. Diagnosing intramedullary schwannoma preoperatively needs high index of suspicion. We are reporting the 10th case of intramedullary schwannoma in the conus region.

Advances in Targeted Therapy for Neurofibromatosis Type 2 (NF2)-Associated Vestibular Schwannomas.

Neurofibromatosis 2 (NF2) is an autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas (VS), meningiomas, ependymomas, spinal and peripheral schwannomas, optic gliomas, and juvenile cataracts. Ongoing studies provide new insight into the role of the NF2 gene and merlin in VS tumorigenesis. As NF2 tumor biology becomes increasingly understood, therapeutics targeting specific molecular pathways have been developed and evaluated in preclinical and clinical studies. NF2-associated VS are a source of significant morbidity with current treatments including surgery, radiation, and observation. Currently, there are no FDA-approved medical therapies for VS, and the development of selective therapeutics is a high priority. This manuscript reviews NF2 tumor biology and current therapeutics undergoing investigation for treatment of patients with VS.

Scoring model to predict postoperative neurological deterioration in spinal schwannoma.

Spinal schwannomas (SSs) are benign tumors affecting the nerve sheath, accounting for 25% of spinal nerve root tumors. Surgery represents the mainstay of treatment for SS patients. Following surgery, approximately 30% of patients experienced developed new or worsening neurological deterioration, which probably represented an inevitable complication of nerve sheath tumor surgery. The objective of this study was to identify the rates of new or worsening neurological deterioration in our center and accurately predict the neurological outcomes of patients with SS by developing a new scoring model. A total of 203 patients were retrospectively enrolled at our center. Risk factors associated with postoperative neurological deterioration were identified by multivariate logistic regression analysis. β-coefficients for independent risk factors were used to define a numerical score to generate a scoring model. The validation cohort at our center was used to verify the accuracy and reliability of the scoring model. Receiver operating characteristic (ROC) curve analysis was used to evaluate the performance of the scoring model. In this study, five measured variables were selected for the scoring model: duration of preoperative symptoms (1 point), radiating pain (2 points), tumor size (2 points), tumor site (1 point), and dumbbell tumor (1 point). The scoring model divided the spinal schwannoma patients into three categories: low risk (0-2 points), intermediate risk (3-5 points), and high risk (6-7 points), with predicted risks of neurological deterioration of 8.7%, 36%, and 87.5%, respectively. And the validation cohort confirmed the model with the predicted risks of 8.6%, 46.4%, and 66.6%, respectively. The new scoring model might intuitively and individually predict the risk of neurological deterioration and may aid individualized treatment decision-making for SS patients.

Spinal Schwannomas: A Proposal for a New Classification to Aid Surgical Planning.

Background The treatment of spinal schwannomas, which is the most common nerve sheath tumor, is total microsurgical resection. The localization, size and relationship with the surrounding structures of these tumors are crucial in terms of preoperative planning. A new classification method is presented in this study for the surgical planning of spinal schwannoma. Methods All patients who underwent surgery for spinal schwannoma between 2008 and 2021 were reviewed retrospectively, along with radiological images, clinical presentation, surgical approach, and postoperative neurological status. Results A total of 114 patients, 57 male, and 57 females were included in the study. Tumor localizations were cervical in 24 patients, cervicothoracic in one patient, thoracic in 15 patients, thoracolumbar in eight patients, lumbar in 56 patients, lumbosacral in two patients, and sacral in eight patients. All tumors were divided into seven types according to the classification method. Type 1 and Type 2 groups were operated on with a posterior midline approach only, Type 3 tumors were operated on with a posterior midline approach and extraforaminal approach, and Type 4 tumors were operated on with only an extraforaminal approach. While the extraforaminal approach was sufficient in type 5 patients, partial facetectomy was required in 2 patients. Combined surgery including hemilaminectomy and extraforaminal approach was performed in the type 6 group. A posterior midline approach with partial sacrectomy/corpectomy was performed in Type 7 group. Conclusion Effective treatment of spinal schwannoma depends on preoperative planning, which includes correctly classifying tumors. In this study, we present a categorization scheme that covers bone erosion and tumor volume for all spinal localizations.