Intramedullary Tumors Research Articles

Intramedullary spinal cord tumors: About 7 cases

This study is about 9 cases of intramedullary spinal cord tumors managed in Neurosurgery Department of UHC AGADIR. There is no sexe predominance and the mean age was 30,8 years. The clinical symptoms is dominated by a spinal cord compression syndrome. All patients underwent spinal MRI. All the patients underwent surgical treatment with total removal in 4 cases, subtotal in 2 cases, and only a biopsy in 1 cases. Histologic examination showed 4 cases of ependymomas, 2 cases of astrocytomas and 1 case of hemangioblastoma. Adjuvant treatment has been administrated in 2 patients. The evolution was good in 4 patients (57,14%) and worsening in 14,28% of the cases.
 Keywords : intramedullary tumors, ependymomas, astrocytomas, hemangioblastomas, surgery

Factors Influencing Surgical Outcomes for Intradural Spinal Tumours: A Single-Centre Retrospective Cohort Study

IntroductionIntradural spinal tumours are relatively uncommon tumours of the central nervous system. In this study, we sought to assess our current practice and determine the factors which affect the surgical outcomes of intradural spinal tumour resection.MethodsAll consecutive patients who underwent surgical resection of intradural spinal tumours from December 2011 to November 2018 were retrospectively reviewed. The Modified McCormick Scale (MMS) was used to grade patients’ neurological status both pre-operatively and at the latest follow-up. The associations between changes in MMS and variables such as patient demographics, tumour location, number and experience of consultants involved in the procedure, use of intraoperative neuro-monitoring, bony spinal exposure and dural closure methods were assessed. A multivariable binary logistic regression model was performed to identify independent predictors of improvements in MMS. All analyses were performed using IBM SPSS 22 (IBM Corp. Armonk, NY), with p<0.05 deemed to be indicative of statistical significance throughout.ResultsA total of 145 patients met the inclusion criteria, with a median age of 56.5 years; of whom 119 had extramedullary tumours and 26 had intramedullary tumours. Methods of dural closure were variable, and there was an increasing trend over time towards using the laminoplasty approach for bony exposure. Neither the experience of consultants (p=0.991) nor the number of consultants involved (p=0.084) was found to be significantly associated with the change in MMS, with the strongest predictor being the baseline MMS (p<0.001). Patients who had adjuvant therapy were also significantly more likely to have a poorer neurological outcome (p=0.001).ConclusionA good neurological baseline is a significant positive predictor of an improved functional outcome. The number and seniority of consultant surgeons involved in intradural spinal tumour resections did not significantly alter the postoperative outcomes of patients in our single-unit retrospective study.

Chapter 13 - 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.

NEUROSURGICAL TREATMENT OF INTRAMEDULLAR TUMOR OF CERVICAL LOCATION

Materials and methods. This report analyzes 47 patients who were operated on at Romodanov Neurosurgery Institute of the Academy of Medical Sciences of Ukraine in the period from 2010 to 2020 due to intramedullary tumors of the cervical spine. Results. All patients with intramedullary tumors of the cervical spine were operated. According to the volume of tumor removal, operations with total (17 observations), subtotal (14 observations), and partial (19 observations) removal were distinguished. The removal of at least 95% of the tumor volume was considered total, which was confirmed by examination of the operating field under a microscope at the end of the operation or by early postoperative MRI. Discussion. Total tumor removal is the gold standard in the surgical treatment of intramedullary spinal cord tumors. Ferrante L. et al reported that none of the patients who underwent total tumor resection had a recurrence, while 57.1% of patients with subtotal resection had a recurrence. The authors argue that the volume of the removed tumor is an important factor influencing the further prognosis. Total tumor resection can be recommended for most cases of intramedullary tumors of the cervical spinal cord. It is not possible to determine preoperatively whether total tumor removal is possible. Conclusions. Intramedullary tumors of the cervical spine can be completely removed. Surgery should be performed immediately after diagnosis without expecting an increase in neurological deficit.

Intramedullary spinal cord tumors in children: the relevance of motor evoked potentials and D-wave monitoring

Intramedullary spinal cord tumors in children: the relevance of motor evoked potentials and D-wave monitoring

Surgery of Intramedullary Spinal Cord Tumors

Surgery of Intramedullary Spinal Cord Tumors

Boxer's mouthguard to facilitate motor evoked potential monitoring during cervical intramedullary tumour excision: Protect and prevent rather than repair and repent!

Intraoperative motor evoked potentials are being increasingly used in surgeries for the removal of spinal tumours. However, this useful monitoring is accompanied by risks such as bite injury to the tongue and oral mucosal soft tissues due to clenching of the teeth and jaws as the patient may not be completely under the effect of neuromuscular blocking agents, resulting in bleeding in the oropharynx. To prevent these complications, we conceptualised and utilised a boxer's mouthguard as a preventive protection during cervical intramedullary tumour excision surgery. Such mouthguards are routinely used in contact sports such as boxing but their clinical application in neuroanaesthesia has not been described. This use is a novel, economical, convenient, standardised and easy way of bite protection and preventing intraoperative tongue, soft-tissue injuries and airway complications.

A case of Intramedullary Tumor Conus Medullas of the Spinal Cord with Drop Foot and Plantar Sensory Disturbance Treadmill Walking Practice in the Early Postoperative Period

A case of Intramedullary Tumor Conus Medullas of the Spinal Cord with Drop Foot and Plantar Sensory Disturbance Treadmill Walking Practice in the Early Postoperative Period

Cranial nerve and intramedullary spinal malignant peripheral nerve sheath tumor associated with neurofibromatosis-1

Background: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon but aggressive neoplasms associated with radiation exposure and neurofibromatosis Type I (NF1). Their incidence is low compared to other nervous system cancers, and intramedullary spinal lesions are exceedingly rare. Only a few case reports have described intramedullary spinal cord MPNST.Case Description: We describe the clinical findings, management, and outcome of a young patient with NF1 who developed aggressive cranial nerve and spinal MPNST tumors. This 35-year-old patient had familial NF1 and a history of optic glioma treated with radiation therapy (RT). She developed a trigeminal MPNST that was resected and treated with RT. Four years later, she developed bilateral lower extremity deficits related to an intramedullary cervical spine tumor, treated surgically, and found to be a second MPNST.Conclusion: To the best of our knowledge, this is the first report of cranial nerve and intramedullary spinal MPNSTs manifesting in a single patient, and only the third report of a confined intramedullary spinal MPNST. This unusual case is discussed in the context of a contemporary literature review.

Maximizing surgical resection in revision surgery for an intramedullary spinal cord tumour using DTI/tractography and direct spinal cord stimulation: A technical note

BackgroundIntramedullary spinal cord tumours are formidable lesions in spine surgery due to the high risk of postoperative deficits associated with surgical resection. Traditional MRI imaging fails to provide a 3-D rendering of the relationship between the tumour and spinal cord sensory and motor pathways; in contrast, DTI/tractography can show this relationship with the potential to reduce surgery-related morbidity. The use of intraoperative somatosensory evoked potentials (SSEP) and transcranial motor evoked potentials (MEP) have also been considered a standard of care to minimize neurologic complications of surgery. However, in situations where both baseline SSEPs and MEPs are unobtainable, D-wave and direct cord stimulation can become invaluable intra-operative tools. MethodsA 6 year old child presented with new growth from an incompletely resected thoracic intramedullary dermoid tumour. Pre-operative DTI/tractography images were obtained. During tumour resection, SSEP and MEP signals were unable to be obtained, however intraoperative monitoring of the spinal cord was undertaken with D-wave monitoring and direct cord stimulation. ResultsThis multimodal monitoring technique allowed identification of the corticospinal tracts intra-operatively and allowed safe tumour resection. Post-operative MRI revealed gross total resection and the patient returned to her pre-operative neurological baseline without incurring any further deficits. ConclusionsWe have presented a technical description of the clinical utility of DTI/tractography in surgical planning, and D-wave monitoring with direct cord stimulation as adjuncts in the resection of an intramedullary cord tumour. This is particularly useful in the scenario where TcMEP/SSEP signals cannot be obtained and in revision surgery for intramedullary spinal cord tumours.

Impact of general anaesthesia on parameters of bulbocavernosus reflex

Dysfunction of the pelvic organs in tumor lesions of the spinal cord is up to 20%. Registration of the bulbocavernosus reflex is performed to assess the integrity of the segmental apparatus of the spinal cord. Polymodal neurophysiological monitoring includes registration of the bulbocavernosus reflex during surgery of spinal cord tumors. The effect of the components of general anesthesia on the parameters of the bulbocavernosus reflex varies significantly according to various medical sources. The aim of the work was to compare the effect of inhalation anesthesia (sevoflurane) and total intravenous anesthesia (propofol) on the parameters of the bulbocavernosus reflex in the surgical treatment of spinal cord tumors. Thirty patients with intradural extramedullary and intramedullary tumors of the spinal cord at the level of Th11–S2 vertebrae were included in the study. The amplitude-frequency parameters of the bulbocavernosus reflex and the threshold intensity of stimulation were assessed in the study. The results of the study demonstrate that intraoperative registration of the bulbocavernosus reflex during resection of tumors of the distal spinal cord can be performed both with inhalation and total intravenous anesthesia. A sustained motor response of the bulbocavernosus reflex requires a higher intensity of stimulation with inhalation anesthesia with sevoflurane than with total intravenous anesthesia. If during the operation the depth of propofol's anesthesia increases by 1 mg/kg/h, then it is necessary to increase the current strength during stimulation by 10–11 mA.

Low grade central osteosarcoma transforming to high grade sarcoma – Clinical implications and brief review of literature !

Low grade central osteosarcoma (LGCO) are rare intramedullary bone tumours of adolescents and young adults with an excellent prognosis. They have a predilection for long bones around the knee joint, but cases have been reported in long bones of arm, tarsals and flat bones of jaw, ribs and scapula as well. Their transformation to high grade sarcomas are rarely seen. We report a case of an 16 years female who presented with complaints of progressive pain in the lower thigh 2 years back. Her x-ray suggested a simple bone cyst in distal femur, following which wide excision of the cyst was done. Histopathology however showed features of LGCO. The patient was apparently alright in the follow up period until 9 months ago when the patient had a recurrence of swelling accompanied with pain at the same site leading to difficulty in walking. This time her X-ray showed neoplastic changes after which the tumor was excised. On microscopy, areas of high grade sarcoma with soft tissue extension were seen predominantly. This case is being reported to make the clinicians and diagnosticians aware of the rare but highly significant implication of transformation of LGCO to high grade tumors as it carries a guarded prognosis and calls for a different treatment strategy compared to conventional LGCO. Low grade central osteosarcoma (LGCO) are rare intramedullary bone tumors seen in adolescents and young adults. It bears an excellent prognosis. We present a case report on LGCO showing transformation to a high grade sarcoma that radically changes its prognosis as well as calls for a different treatment strategy.

Clinical Prediction Modeling in Intramedullary Spinal Tumor Surgery.

Artificial intelligence is poised to influence various aspects of patient care, and neurosurgery is one of the most uprising fields where machine learning is being applied to provide surgeons with greater insight about the pathophysiology and prognosis of neurological conditions. This chapter provides a guide for clinicians on relevant aspects of machine learning and reviews selected application of these methods in intramedullary spinal cord tumors. The potential areas of application of machine learning extend far beyond the analyses of clinical data to include several areas of artificial intelligence, such as genomics and computer vision. Integration of various sources of data and application of advanced analytical approaches could improve risk assessment for intramedullary tumors.

Clinically aggressive pediatric spinal ependymoma with novel MYC amplification demonstrates molecular and histopathologic similarity to newly described MYCN-amplified spinal ependymomas

Primary spinal cord tumors contribute to ≤ 10% of central nervous system tumors in individuals of pediatric or adolescent age. Among intramedullary tumors, spinal ependymomas make up ~ 30% of this rare tumor population. A twelve-year-old male presented with an intradural, extramedullary mass occupying the dorsal spinal canal from C6 through T2. Gross total resection and histopathology revealed a World Health Organization (WHO) grade 2 ependymoma. He recurred eleven months later with extension from C2 through T1-T2. Subtotal resection was achieved followed by focal proton beam irradiation and chemotherapy. Histopathology was consistent with WHO grade 3 ependymoma. Molecular profiling of the primary and recurrent tumors revealed a novel amplification of the MYC (8q24) gene, which was confirmed by fluorescence in situ hybridization studies. Although MYC amplification in spinal ependymoma is exceedingly rare, a newly described classification of spinal ependymoma harboring MYCN (2p24) amplification (SP-MYCN) has been defined by DNA methylation-array based profiling. These individuals typically present with a malignant progression and dismal outcomes, contrary to the universally excellent survival outcomes seen in other spinal ependymomas. DNA methylation array-based classification confidently classified this tumor as SP-MYCN ependymoma. Notably, among the cohort of 52 tumors comprising the SP-MYCN methylation class, none harbor MYC amplification, highlighting the rarity of this genomic amplification in spinal ependymoma. A literature review comparing our individual to reported SP-MYCN tumors (n = 26) revealed similarities in clinical, histopathologic, and molecular features. Thus, we provide evidence from a single case to support the inclusion of MYC amplified spinal ependymoma within the molecular subgroup of SP-MYCN.

척수 종양 수술 중 척수 지도화

The main treatment for most patients with intramedullary spinal cord tumors is surgical resection. Intraoperative neurophysiological monitoring can be applied in this case to reduce the risk for the occurrence of postoperative neurological complications. In addition to motor-evoked, somatosensory-evoked potentials and the bulbocavernosus reflex, which are applied in various spinal surgery types, ‘spinal cord mapping (SCM)’ can be used for intramedullary spinal cord tumor surgery. Two examples are the dorsal column mapping before myelotomy to determine the electrophysiological midline, to attempt minimizing the postoperative dorsal column damage, and motor tract mapping to reduce the risk of motor-pathway damage during tumor removal. Our review explores the research published overseas regarding these two mapping methods, including technical aspects and key points in the mapping application. Although SCM has not yet been established in Korea, its application is expected to increase soon.

척수 수술에서 수술 중 신경계 감시

The recent breakthrough development of the intraoperative neurophysiological monitoring (IONM) has greatly contributed to minimizing neural damage and making surgery safe. In particular, IONM can significantly improve the patient’s prognosis in the case of spinal cord surgery with frequent post-operative neural damage. Somatosensory evoked potential and motor evoked potential are highly recommended for IONM in the spinal cord surgery, and spinal cord mapping plays an important role in intramedullary tumor surgery. In this review, we will discuss the modalities, methods, and values of IONM in spinal cord surgery, and consider the future direction of IONM development.

Non-dysraphic Subpial Intramedullary Lipoma of Cervicothoracic Spine

Intramedullary spinal cord tumors are rare and comprise less than one percentage of all central nervous system (CNS) neoplasms. When encountered, most are found in the lumbosacral region with coinciding spinal dysraphism. Here, we discuss a case of isolated non-dysraphic intramedullary lipoma of cervicothoracic spine in an adolescent who was surgically managed with posterior decompression and subtotal resection via laminoplasty. A 21-year-old male adolescent, without a history of spinal dysraphism, presented with a progressive sensory ataxia. This manifestation was attributed to magnetic resonance documented intramedullary lipoma, extending from C7 to T2 spinal level. Patient underwent subtotal surgical resection of the lesion; and histopathology report was confirmed as spinal lipoma. Surgical management of this rare pathology has a wide variety of options depending on clinical presentation. In our case, a subtotal resection and laminoplasty was achieved with no further worsening of neurologic symptoms.

Lentiviral-Induced Spinal Cord Gliomas in Rat Model.

Intramedullary spinal cord tumors are a rare and understudied cancer with poor treatment options and prognosis. Our prior study used a combination of PDGF-B, HRAS, and p53 knockdown to induce the development of high-grade glioma in the spinal cords of minipigs. In this study, we evaluate the ability of each vector alone and combinations of vectors to produce high-grade spinal cord gliomas. Eight groups of rats (n = 8/group) underwent thoracolumbar laminectomy and injection of lentiviral vector in the lateral white matter of the spinal cord. Each group received a different combination of lentiviral vectors expressing PDGF-B, a constitutively active HRAS mutant, or shRNA targeting p53, or a control vector. All animals were monitored once per week for clinical deficits for 98 days. Tissues were harvested and analyzed using hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining. Rats injected with PDGF-B+HRAS+sh-p53 (triple cocktail) exhibited statistically significant declines in all behavioral measures (Basso Beattie Bresnahan scoring, Tarlov scoring, weight, and survival rate) over time when compared to the control. Histologically, all groups except the control and those injected with sh-p53 displayed the development of tumors at the injection site, although there were differences in the rate of tumor growth and the histopathological features of the lesions between groups. Examination of immunohistochemistry revealed rats receiving triple cocktail displayed the largest and most significant increase in the Ki67 proliferation index and GFAP positivity than any other group. PDGF-B+HRAS also displayed a significant increase in the Ki67 proliferation index. Rats receiving PDGF-B alone and PDGF-B+ sh-p53 displayed more a significant increase in SOX2-positive staining than in any other group. We found that different vector combinations produced differing high-grade glioma models in rodents. The combination of all three vectors produced a model of high-grade glioma more efficiently and aggressively with respect to behavioral, physiological, and histological characteristics than the rest of the vector combinations. Thus, the present rat model of spinal cord glioma may potentially be used to evaluate therapeutic strategies in the future.

Syringomyelia From Extramedullary Compression: Resolution After Microsurgical Resection of a High-Cervical Spine Schwannoma: 2-Dimensional Operative Video.

Syringomyelia with compression at the foramen magnum is a well-studied subject with a pathophysiological hypothesis explaining syrinx development and treatment.1,2 However, compression of the cervical cord, as in cervical spondylosis, can also lead to cavitation in the spinal cord.3,4 Although the association of intramedullary spinal cord tumors with syringomyelia is well known,5 the association of extramedullary tumors with syringomyelia is scarcely reported, and it is of unknown mechanism and uncertain outcomes regarding syrinx resolution. Syringomyelia is reported to be associated with intramedullary spinal schwannoma.6 However, spinal schwannomas are by and large extramedullary tumors that produce deficits through compressive radiculopathy or myelopathy. The association of extramedullary schwannomas with syringomyelia is extremely rare. In this article, we present a patient with an extramedullary high-cervical schwannoma causing significant syringomyelia who underwent microsurgical resection of the schwannoma, with subsequent total resolution of the syringomyelia. The patient consented for surgery. Images from Lancet 1969 at 6:36 in video Reprinted from The Lancet, Vol. 294, Bernard Williams, The Distending Force in the Production of "Communicating Syringomyelia," Pages 189-193, Copyright 1969, with permission from Elsevier.

Analysis of Factors Influencing the Long-term Outcome in Primary Spinal Cord Tumors: Review of Literature

Abstract Introduction Primary spinal cord tumors are amenable to total surgical excision but the outcomes are still diverse. Objective The purpose of this study is to identify the significant factors which influence the long-term outcome of spinal cord tumors. Materials and Methods A total of 84 consecutive spinal cord tumor patients who underwent microsurgical excision during April 2003 to 2014 were retrospectively studied. Patients operated after April 2014 were not considered. Ten factors were taken and correlated with the outcome. Multiple regression analysis was used. Patients were periodically evaluated at 1, 6 months, 1, 2, and 5 years. In 72 cases, postoperative MRI scans were performed. The follow-up period ranged between 5 to 15 years. Results In our study, we had 64 extramedullary tumors and 21 intramedullary tumors with varied pathology. Axial location of the tumour (20.294/0.000), extent of the resection of the tumour (13.827/0.001), preoperative Nurick grade (11.349/0.023), and location of the tumour in the spine with respect to vertebral segments (8.151/0.017) were significant predictive factors. We had good outcomes in 65 cases and poor outcomes in 19 cases. Conclusion Our results show location of the tumor with respect to axial plane and vertebral segments were the main contributing factors. They influence the extent of excision of the spinal cord tumor. Preoperative neurological status is the fourth factor which independently determines the long-term outcome. It is one of the few papers where patients have been followed-up for a long period ranging from 5 to 15 years.