Intramedullary Spinal Cord Tumors: Whole-Genome Sequencing to Assist Management and Prognosis.

Intramedullary spinal cord tumors (IMSCTs) harbor unique genetic mutations which may play a role in prognostication and management. To this end, we present the largest cohort of IMSCTs with genetic characterization in the literature from our multi-site institutional registry. A total of 93 IMSCT patient records were reviewed from the years 1999 to 2020. Out of these, 61 complied with all inclusion criteria, 14 of these patients had undergone genetic studies with 8 undergoing whole-genomic sequencing. Univariate analyses were used to assess any factors associated with progression-free survival (PFS) using the Cox proportional hazards model. Firth's penalized likelihood approach was used to account for the low event rates. Fisher's exact test was performed to compare whole-genome analyses and specific gene mutations with progression. PFS (months) was given as a hazard ratio. Only the absence of copy neutral loss of heterozygosity (LOH) was shown to be significant (0.05, p = 0.008). Additionally, higher risk of recurrence/progression was associated with LOH (p = 0.0179). Our results suggest LOH as a genetic predictor of shorter progression-free survival, particularly within ependymoma and glioblastoma tumor types. Further genomic research with larger multi-institutional datasets should focus on these mutations as possible prognostic factors.

A multilevel posterior tension band-sparing laminectomy for intraspinal lesions: patient series.

BACKGROUND: Minimally invasive spine surgery (MISS) represents a major development in spinal tumor surgery. However, considering that many intradural lesions compromise multiple spinal segments, MISS has certain limitations. Thus, some intraspinal lesions still require traditional approaches. Because laminectomy has been shown to predispose patients to kyphosis, laminoplasty and hemilaminectomy are the most widely used approaches to preserve the posterior tension band (PTB). However, these techniques are not devoid of complications. To overcome these issues, the authors modified a previously described technique to preserve the PTB while removing various types of intradural lesions. This procedure was originally designed to treat lumbar stenosis and was modified to avoid muscle ischemia during long procedures. OBSERVATIONS: Between 2014 and 2021, the authors found 17 cases of spinal lesions with a minimum of 2 years of follow-up after surgical treatment using their approach. No significant postoperative changes in the paraspinal Goutallier grade or spinal angles were observed. The cross-sectional area of the measured paraspinal muscles decreased 6% postoperatively. By performing certain technical modifications in this PTB-sparing (PBS) laminectomy, the authors avoided ipsilateral muscle ischemia. LESSONS: In this initial series, PBS laminectomy proved to be a safe, versatile, inexpensive, and reliable technique to remove intraspinal lesions.

Postoperative neurological complications in intradural extramedullary tumors: A 10-year experience of a single center.

BACKGROUND: Intradural extramedullary spinal cord tumors (IDEMs) cause neurological symptoms due to compression of the spinal cord and caudal nerves. The purpose of this study was to investigate the incidence of postoperative neurological complications after surgical resection of IDEM and to identify factors associated with such postoperative neurological complications. METHODS: We retrospectively analyzed 85 patients who underwent tumor resection for IDEM between 2010 and 2020. We investigated the postoperative worsening of neurological disorders. The patients were divided into two groups: those with and without postoperative neurological complications. Patient demographic characteristics, tumor level, histological type, and surgery-related factors were also compared. RESULTS: The mean age at the time of surgery was 57.4 years, and histological analysis revealed 45 cases of schwannoma, 34 cases of meningioma, three cases of myxopapillary ependymoma, one case of ependymoma, one case of hemangioblastoma and one case of lipoma. There were five cases (5.8%) of postoperative neurological complications, and four patients improved within 6 months after surgery, and one patient had residual worsening. There were no statistically significant differences in age, sex, tumor location, preoperative modified McCormick Scale grade, histology, tumor occupancy, or whether fixation was performed in the presence or absence of postoperative neurological complications. All four cases of meningioma with postoperative neurological complications had preoperative neuropathy and meningiomas were located in the anterior or lateral thoracic spine. CONCLUSIONS: Neurological complications after surgical resection for IDEM occurred in 5.8% of patients. Meningiomas with postoperative neurological complications located anteriorly or laterally in the thoracic spine.

Laminectomy with Laminar Reconstruction for Resection of Intradural Tumors at the Thoracolumbar Junction: A Technical Note.

OBJECTIVE: To introduce a new laminar reconstruction technique to treat primary spinal cord tumors. METHODS: Laminectomy and laminoplasty techniques have been used to treat intradural spinal tumors. The advantage of laminectomy is its superior exposure of the spinal cord, whereas the advantage of laminoplasty is the reconstruction of the dorsal roof of the spine. In this technical note, we present a technique that combines a full laminectomy to maximize exposure, with a reconstructive technique to repair the lamina. This technique restores the posterior ligamentous complex to preserve spinal biomechanics. RESULTS: In this illustrative case, a 55-year-old woman with severe back pain radiating to the right lower extremity was found to have an intradural tumor at the T12-L1 spinal level. Given the transitional level of the spine and potentially high biomechanical stresses on the posterior support structures, we used a T12 laminectomy to resect the tumor, followed by reconstruction using miniplates. The patient tolerated the surgery well, without any complications. She was discharged home and was doing well during the 3 months follow-up visit. Appropriate patient consent was obtained. CONCLUSIONS: Laminectomy and laminar reconstruction allow maximum visualization and manipulation of the tumor, followed by restoration of the dorsal roof of the spinal ring, and is an effective technique for treating spinal cord tumors.

Surgical Management of Multiple-Level Lumbar Spinal Schwannomas: A Case Report.

The increase in benign spinal tumors among adults over the last decade has been a major cause of concern. This worrisome trend has been attributed to many factors, including improved detection techniques, enhanced access to medical care, and the aging population. The research primarily focuses on Schwannoma, which is a rare type of tumor that arises from Schwann cells, which are responsible for producing the myelin sheath that surrounds and protects nerves. Although the majority of schwannomas are benign, there have been instances where they have transformed into malignant tumors, potentially leading to significant morbidity and mortality. We report a case of a 68-year-old woman who presented with progressive back pain and weakness in both lower limbs over the past months. The pain was initially localized to the lower back but gradually intensified and radiated down to the legs. The patient reported difficulty walking and a sensation of tingling and numbness in the feet. She denied any recent trauma or significant medical history. On physical examination, there was reduced muscle strength (3/5) in both lower limbs. The patient exhibited hyporeflexia in the knees and ankle. A magnetic resonance imaging (MRI) of the spine was performed, revealing a well-defined mass lesion located in the lumbar region, compressing the spinal cord from L2 to L5. The patient was counseled and prepared for surgical resection of the tumor. Histopathological findings revealed features of peripheral nerve sheath tumors and cellular schwannomas. The patient recovered well postoperatively. The surgeon operating should be mindful of the potential presence of a mobile schwannoma, even though it is rarely mentioned in the literature. Being aware of this possibility can help prevent unnecessary surgical dissection, which can lead to higher rates of complications and morbidity. Although it is plausible that this case could have involved a mobile schwannoma, there was not enough evidence to support it as we performed a laminectomy on multiple levels due to the tumor's size.

Effect of race, sex, and socioeconomic factors on overall survival following the resection of intramedullary spinal cord tumors.

INTRODUCTION: Intramedullary spinal cord tumors (IMSCTs) account for 2-4% of all primary CNS tumors. Given their low prevalence and the intricacy of their diagnosis and management, it is critical to address the surrounding racial and socioeconomic factors that impact the care of patients with IMSCTs. This study aimed to investigate the association between race and socioeconomic factors with overall 5 year mortality following the resection of IMSCTs. METHODS: The study used the National Cancer Database to retrospectively analyze patients who underwent resection of IMSCTs from 2004 to 2017. Patients were divided into four cohorts by race/ethnicity, facility type, insurance, median income quartiles, and living area. The primary outcome of interest was 5 year survival, and secondary outcomes included postoperative length of stay and 30 day readmission. Descriptive and multivariable analyses were used to identify independent factors associated with mortality, with statistical significance assessed at a 2-sided p < 0.05. RESULTS: We evaluated the patient characteristics and outcomes for 8,028 patients who underwent surgical treatment for IMSCTs between 2004 and 2017. Most patients were white males (52.4%) with a mean age of 44 years where 7.17% of patients were Black, 7.6% were Hispanic, and 3% were Asian. Most were treated in an academic/research program (72.4%) and had private insurance (69.2%). Black patients had a higher odd of 5 year mortality (OR 1.4; 95% CI 1.1 to 1.77; p = 0.04) compared to white patients, while no significant differences in mortality were observed among other races. Factors associated with lower odds of mortality included being female (OR 0.89; 95% CI 0.78 to 1.02; p < 0.01), receiving treatment in an academic/research program (OR 0.51; 95% CI 0.33 to 0.79; p = 0.04), having private insurance (OR 0.65; 95% CI 0.45 to 0.93; p = 0.02), and having higher income quartiles (OR 0.77; 95% CI 0.62 to 0.96; p = 0.02). CONCLUSION: Our study sheds light on the healthcare disparities that exist in the surgical management of IMSCTs. Our findings indicate that race, sex, socioeconomic status, and treatment facility are independent predictors of 5 year mortality, with Black patients, males, those with lower socioeconomic status, and those treated at non-academic centers experiencing significantly higher mortality rates. These alarming disparities underscore the urgent need for policymakers and researchers to address the underlying factors contributing to these discrepancies and provide equal access to high-quality surgical care for patients with IMSCTs.

Non-dysraphic intramedullary spinal cord lipoma of the child: Report of 3 cases.

BACKGROUND AND PURPOSE: Non-dysraphic intramedullary spinal cord lipomas (NDSCL) represent 1% of spinal cord tumors. They are less frequent than dysraphic spinal cord lipomas and clinical presentation is unspecific. There are no guidelines on surgical management. MATERIAL AND METHODS: We report three observations of NDSCL in children, focusing on the clinical presentation, surgical management and postoperative outcome. RESULTS: The patients, one female and two males, aged from 5 months to 10 years presented with neurological deterioration, pain, spinal rigidity and in two cases, a subcutaneous mass. Spinal MRI found intradural lipomas without spina bifida, located in the cervico-thoracic area in all cases. The lipoma extended to the medulla oblongata in two cases and was in the lumbar region in the third. These lipomas were massive, requiring decompression surgery. Surgery confirmed the lipoma to be subpial. We performed debulking of the lipoma without attempting total resection, and with or without dural plasty and laminoplasty, followed by minerva cast in two cases, and avoidance of standing in the youngest. Satisfactory recovery occurred in all three cases. After a follow-up between 4 months and 9 years, the outcome was favorable in all cases, and no patient presented with secondary spinal deformation or lipoma progression. CONCLUSION: NDSCL is a rare entity, which often manifests with progressive pain and neurological deficits. In our experience, partial resection with or without dural plasty and laminoplasty has been associated with satisfactory postoperative outcomes and no recurrence of symptoms. We should be attentive to the risk of postoperative spinal deformity in these young patients.

Dorsal column mapping in resection of intramedullary spinal cord tumors: a prospective comparison of two methods and neurological follow-up.

PURPOSE: In surgery for intramedullary spinal cord tumors (imSCT), distortion of the anatomy challenges the visual identification of dorsal columns (DC) for midline myelotomy. Dorsal column mapping (DCM) and spinal cord stimulation (SCS) can identify DC neurophysiologically. We compare application and feasibility of both methods. METHODS: Patients with surgically treated imSCT were prospectively included between 04/2017 and 06/2019. The anatomical midline (AM) was marked. SSEPs at the DC after stimulation of tibial/median nerve with an 8-channel DCM electrode and cortical SSEP phase reversal at C3/C4 after SCS using a bipolar concentric probe were recorded. Procedural and technical aspects were compared. Standardized neurological examinations were performed preoperatively, 1 week postoperatively and after more than 12 months. RESULTS: The DCM electrode detected the midline in 9/13 patients with handling limitations in the remaining patients. SCS was applicable in all patients with determination of the midline in 9/13. If both recordings could be acquired (6/13), concordance was 100%. If baseline SSEPs were poor, both methods were limited. SCS was less time-consuming (p = 0.001), cheaper, and easier to handle. In 92% of cases, the AM and neurophysiologic midlines were concordant. After myelotomy, 3 patients experienced > 50% reduction in amplitude of SSEPs. Despite early postoperative worsening of DC function, long-term follow-up showed significant recovery and improvement in quality of life. CONCLUSION: DCM and SCS may help confirm and correct the AM for myelotomy in imSCT, leading to a favorable long-term neurological outcome in this cohort. SCS evolved to be superior concerning applicability, cost-effectiveness, and time expenditure.

Current Applications of Machine Learning for Spinal Cord Tumors.

Spinal cord tumors constitute a diverse group of rare neoplasms associated with significant mortality and morbidity that pose unique clinical and surgical challenges. Diagnostic accuracy and outcome prediction are critical for informed decision making and can promote personalized medicine and facilitate optimal patient management. Machine learning has the ability to analyze and combine vast amounts of data, allowing the identification of patterns and the establishment of clinical associations, which can ultimately enhance patient care. Although artificial intelligence techniques have been explored in other areas of spine surgery, such as spinal deformity surgery, precise machine learning models for spinal tumors are lagging behind. Current applications of machine learning in spinal cord tumors include algorithms that improve diagnostic precision by predicting genetic, molecular, and histopathological profiles. Furthermore, artificial intelligence-based systems can assist surgeons with preoperative planning and surgical resection, potentially reducing the risk of recurrence and consequently improving clinical outcomes. Machine learning algorithms promote personalized medicine by enabling prognostication and risk stratification based on accurate predictions of treatment response, survival, and postoperative complications. Despite their promising potential, machine learning models require extensive validation processes and quality assessments to ensure safe and effective translation to clinical practice.

Flank Pain as a First Symptom of a Diffuse Midline Glioma.

Diffuse midline gliomas are a new entity in the WHO Classification of Tumors of the Central Nervous System, corresponding to grade 4 gliomas. The diagnostic pathognomonic feature is the presence of a H3K27M mutation. Although mainly seen in children, cases in adults have also been reported. The symptoms are highly variable and usually dependent on the location and extent of spinal cord compression.

Brain and Spinal Cord Tumors Among the Life-Threatening Health Problems: An Introduction.

As one of the global concerns, cancers, including brain and spinal cord tumors, are responsible for mortalities and irreversible morbidities in the affected patients. Although advancements in molecular pathology and imaging of tumors may have influenced the incidence rate due to higher diagnosis in early stages, exposure to environmental risk factors could be another explanation for increased incidence of these tumors over the past decades. Similar to many other tumors, the CNS tumors begin in cellular dimension with activation of different molecular pathways. Several genetic, epigenetic, and immunologic pathways and processes are already discovered to play roles in pathophysiology of these tumors, which mostly will eventually become symptomatic. Each of these tumors may exhibit imaging characteristics, making it possible to list a series of differential diagnosis before histopathologic examination. Advances in molecular pathology have resulted in better understanding and categorization of CNS tumors, leading to better decision-making on the most appropriate therapeutic approach for each category, as well as proposing new therapeutic modalities to treat these tumors. As an introduction to the 2-volume book, this chapter addressed different types of human brain and spinal cord tumors based on the fifth version of WHO classification of CNS tumors.

The Role of Neuro-Inflammation and Innate Immunity in Pathophysiology of Brain and Spinal Cord Tumors.

Inflammation and innate immune system play a central role in cancers, including those affecting the central nervous system (CNS). Currently, classification of neoplasms, especially regarding gliomas, is established on molecular mutations in isocitrate dehydrogenase (IDH) genes and the presence of co-deletion 1p/19q. Treatment, in most of brain and spinal cord tumors, is centered on surgery, radiotherapy and pharmacological approaches with chemotherapeutic agents. However, the results of the treatments, after several decades, are not completely satisfactory. Cytokines and angiogenic factors are closely linked to the brain cancer behavior. Moreover, recent studies suggest a link between inflammation and tumorigenesis, underlying the complex nature of this topic, especially the anti- and pro-tumoral activities of inflammation and the two-way interactions between immune and tumor cells. The current understanding of the mechanisms by which CNS cancer cells modulate the immune system, especially how bi-directional communications between immune cells and tumor cells create an immunosuppressed microenvironment, gives important information about the promotion of tumor survival and growth. Here, we have briefly reviewed the current literature on this topic, focusing on the possible role of inflammation and innate immunity involved in the origin and in the development of CNS tumors.

The Role of Cellular Immunity and Adaptive Immunity in Pathophysiology of Brain and Spinal Cord Tumors.

Major advances have been made in our understanding of CNS tumors, especially glioma, however, the survival of patients with malignant glioma remains poor. While radiation and chemotherapy have increased overall survival, glioblastoma multiforme (GBM) still has one of the worst 5-year survival rates of all human cancers. Here, in this chapter, the authors review the abrogation of the immune system in the tumor setting, revealing many plausible targets for therapy and the current immunotherapy treatment strategies employed. Notably, glioma has also been characterized as a subset of primary spinal cord tumor and current treatment recommendations are outlined here.

Surgical outcomes in instrumented surgery for dumbbell type spinal cord tumor -the comparison with non-instrumented surgery for spinal cord tumor.

BACKGROUND: There is little information on outcomes for spinal cord tumor treated surgically with instrumentation. Analysis of surgical outcomes and complications in such cases is needed to develop generalizable conclusions and to help inform patients. METHODS: The subjects were 41 patients treated with instrumentation surgery for dumbbell type tumor resection. Demographic data; tumor histology, level, and location; number of fused vertebra; use of a bilateral or hemilateral screw; operative time; EBL; TcMEP monitoring; lumbar subarachnoid drainage; duration of subfascial drainage; postoperative motor and sensory deficits; CSF leakage, implant-related complications; time for union of fused vertebra; salvage surgeries, and pre-/postoperative McCormick scale were obtained from medical records. Significant factors related to postoperative motor deficits were identified. RESULTS: Postoperative motor deficit occurred in 9 cases (22.0%) and all recovered in 30 days after surgery. CSF leakage at 7 days and 2 years after surgery was subfascial (n = 31, n = 6) and subcutaneous (n = 3, n = 4). Cases with postoperative motor deficits more commonly had lower cervical lesions; those with CSF leakage had longer operative times; and those with delayed union had more use of hemilateral instrumentation. CONCLUSION: In this study in 41 spinal cord tumors treated surgically with instrumentation, the rate of postoperative motor deterioration was 22.0%, and CSF leakage was found in 17.1%.

Intraoperative neurophysiology in intramedullary spinal cord tumor surgery.

Intramedullary spinal cord tumor (ISCT) surgery is challenged by a significant risk of neurological injury. Indeed, while most ISCT patients arrive to surgery in good neurological condition due to early diagnosis, many experience some degree of postoperative sensorimotor deficit. Thus, intraoperative neuromonitoring (IONM) is invaluable for providing functional information that helps neurosurgeons tailor the surgical strategy to maximize resection while minimizing morbidity. Somatosensory evoked potential (SEP), muscle motor evoked potential (mMEP), and D-wave monitoring are routinely used to continuously assess the functional integrity of the long pathways within the spinal cord. More recently, mapping techniques have been introduced to identify the dorsal columns and the corticospinal tracts. Intraoperative SEP decline is not a sufficient reason to abandon surgery, since SEPs are very sensitive to anesthesia and surgical maneuvers. Yet, a severe proprioceptive deficit may adversely impact daily life, and the value of SEPs should be reconsidered. While mMEPs are good predictors of short-term motor outcome, the D-wave is the strongest predictor of long-term motor outcome, and its preservation during surgery is essential. Mapping techniques are promising but still need validation in large cohorts of patients to determine their impact on clinical outcome. The therapeutic rather than merely diagnostic value of IONM in spine surgery is still debated, but there is emerging evidence that IONM provides an essential adjunct in ISCT surgery.

Comparing Two Improved Techniques With the Traditional Surgical Techniques for Intra and Extramedullary Spinal Tumor Resection: A Report of 280 Cases.

Objectives: Spinal tumors remain a challenging problem in modern neurosurgery. The high rate of postoperative morbidity associated with intramedullary tumors makes the need for safer surgical techniques invaluable. This study analyses our experience with the treatment of spinal cord tumors and compares traditional management and a new different surgical approach to intramedullary tumors with an associated hydrosyringomyelia. Materials and Methods: This retrospective study compared standard surgical techniques and 2 newer modified techniques for intra and extramedullary spinal tumors at the Neurosurgery center for spinal cord tumors of the Republic of Uzbekistan. Preoperative neurological status was recorded with the ASIA/ISNCSCI scale. Postoperative outcome was graded using the Nurrick score. Results: Of the 280 cases, there were 220 (78.5%) extramedullary and 60 (21.5%) with intramedullary spinal tumors. The control and main group had 159 (56.8%) and 121 (43.2%) patients, respectively. Severe compression myelopathy (ASIA- A, B, C) was 217 (77.5%) patients i.e., ASIA A-39 (13.9%); B-74 (26.4%), and C-104 (37.1%). In 74 extramedullary tumors (33.6%) treated with the new method, good postoperative outcomes in 44 cases (59.5%) with OR = 1.9; 95% CI 1.1-3.3 (p < 0.05). Thirty-seven (61.7%) intramedullary tumors were treated with the newer modified technique. There was no difference with the standard method (p = 0.15). However, when comparing postoperative Nurick grade 1-2 with grade 3-4, the newer strategy was superior with improvement in 24 (65%) patients, OR = 3.46; 95% CI 1.2-10.3 (p < 0.05). Conclusion: When compared with standard methods, the proposed newer modified strategy of surgical treatment of spinal cord tumors with the insertion of a syringosubarachnoid shunt in the presence of an associated hydrosyringomyelia is associated with better postoperative outcome (Nurick 1 and 2) in 64.8%.

Relevance of intraoperative motor evoked potentials and D-wave monitoring for the resection of intramedullary spinal cord tumors in children.

The purpose of this study was to evaluate the reliability of motor evoked potentials (MEP), somatosensory evoked potentials (SSEP), and D-wave monitoring as predictors of postoperative neurological deficits in pediatric patients undergoing resection of intramedullary spinal cord tumors (IMSCTs). Additionally, we aimed to determine whether alerts in the intraoperative neurophysiological monitoring (IONM) influenced the extent of resection (EOR). A retrospective analysis of the pediatric patients who underwent resection of IMSCT between March 2010 and April 2021 with an IONM guidance was performed. IONM alerts were recorded and correlated with patients' clinical status at discharge. Twenty three pediatric patients were included. MEP and SSEP were successfully elicited in all patients, while D-wave monitoring was feasible for 14 of them (60.9%). Significant IONM alerts occurred in 6 individuals (26.1%) with monitorable MEP and SSEP and 2 patients with monitorable D-waves (14.3%). The sensitivity, specificity, positive predictive value, and negative predictive value accounted for 100%, 81.8%, 20%, 100% for MEP, 100%, 92.3%, 50%, 100% for D-wave, and 50%, 81%, 20%, 94.44% for SSEP, respectively. Both MEP (p < 0.001) and D-wave monitoring (p < 0.001) accurately predicted postoperative motor deficits, while SSEP failed to provide significant accuracy regarding sensory deficits (p = 0.491). Gross-total tumor resection was performed in 29.4% of patients without IONM alerts and 33.3% of patients with IONM alerts, indicating that IONM alerts did not limit the EOR (p = 0.0857). MEP and D-wave monitoring can be perceived as reliable IONM modalities in pediatric IMSCTs surgery. Caution is needed with the implementation of SSEP to guide surgical decisions.

Two Cases of Primary Spinal Glioblastoma in Adults Treated With Multimodality Therapy.

Primary spinal glioblastoma (GBM) is a rare disease entity with no established standard treatment. We present two cases of primary spinal GBM initially presenting with motor-sensory deficits and back pain. Management varied in that the first patient received subtotal resection followed by radiation therapy, while the second patient underwent gross total resection followed by radiation therapy and temozolomide. The first patient died from hypoxemia secondary to disease progression affecting diaphragmatic motion three months after diagnosis. The second patient progressed intracranially and died 7.4 months after diagnosis. There is no standard of care for primary spinal GBM, so treatment should follow a multidisciplinary discussion focused on patient-specific goals. These cases highlight the poor prognosis of primary spinal GBM despite different treatment approaches, necessitating accurate reporting of all similar cases to help improve knowledge and management of this rare malignancy.