A novel lateral myelotomy approach for the treatment of lateral or ventrolateral spinal gliomas.

OBJECTIVE: To describe a novel surgical approach in which myelotomy was performed lateral to the dorsal root entry zone (LDREZ), for the treatment of lateral or ventrolateral spinal intramedullary glioma. METHODS: This study reviewed six patients with lateral or ventrolateral spinal intramedullary glioma who received surgical treatments by using myelotomy technique of LDREZ approach. The patient's clinical characteristics, magnetic resonance imaging (MRI) results, and follow-up outcomes were analyzed. The neurological function of patients before and after operation was assessed based on the Frankel scale system. The anatomical feasibility, surgical techniques, advantages and disadvantages of LDREZ approach were analyzed. RESULTS: Myelotomy technique of LDREZ approach was employed in all 6 patients. Gross total resections were achieved in 4 patients, and 2 patients with astrocytoma (case 2, 6) underwent partial removal. The perioperative recovery was all smooth and all the patients were discharged on schedule. All the patients who suffered from neuropathic pain were relieved. After surgery, neurological function remained unchanged in 3 patients. 2 patients improved from Frankel grade B to C, and 1 patient deteriorated from Frankel grade D to C immediately after surgery and returned to Frankel grade D at 3 months follow-up. Regarding to the poor prognosis of high-grade glioma, the two cases with WHO IV glioma didn't achieve long survival. CONCLUSION: LDREZ approach is feasible and safe for the surgical removal of lateral or ventrolateral spinal gliomas. This approach can provide a direct pathway to lateral or ventrolateral spinal gliomas with minimal damage to normal spinal cord.

Simultaneous L1-2 Bulged Disc and Mobile Spinal Schwannoma Causing Cauda Equina Syndrome: A Rare Case Report.

BACKGROUND Aside from the rarity of mobile spinal schwannomas, the coexistence of these tumors with herniated intervertebral disc is also scarce. Furthermore, cauda equina syndrome (CES), as a manifestation of intraspinal schwannomas has been reported rarely. Described here is a case of simultaneous lumbar disc bulge and mobile spinal schwannoma presented with intermittent symptoms of CES. CASE REPORT A 62-year-old man presented with severe but intermittent leg pain for 2 weeks, which later progressed to an episode of lower extremity weakness and difficulty in urination. Magnetic resonance imaging revealed an intraspinal tumor that moved in position relative to the L1-2 disc bulge on scans 6 h apart, with associated spontaneous regression in symptoms. The tumor was found to be a mobile spinal schwannoma, originated from a nerve root. A standard microdissection technique was used to remove the tumor through a spinous process-sparing unilateral approach, with complete laminectomy of L1. Use of intraoperative ultrasound facilitated the accurate tumor localization. Postoperatively, the patient no longer had symptoms. CONCLUSIONS This report presents a combination of a common spinal pathology, intervertebral disc herniation, alongside a rare condition, mobile spinal schwannoma, whose uncommon clinical manifestations, such as CES can cause irreversible neurological deficits. Surgeons need to remain vigilant of potential atypical scenarios when treating patients. Surgical treatment challenges regarding the mobility of tumors, such as accurate localization, should be addressed using intraoperative imaging to avoid wrong-level surgery. To mitigate the irreversible neurological complications, patients should receive comprehensive information for alarming signs of CES.

Characterizing the presentation, management, and clinical outcomes of patients with intradural spinal chordomas: a systematic review.

OBJECTIVE: Chordomas are locally aggressive neoplasms of the spine or skull base that arise from embryonic remnants of the notochord. Intradural chordomas represent a rare subset of these neoplasms, and few studies have described intradural chordomas in the spine. This review evaluates the presentation, management, and outcomes of intradural spinal chordomas. METHODS: A systematic review of PubMed/MEDLINE, EMBASE, Cochrane Library, Scopus, and Web of Science was performed. Studies describing at least 1 case of intradural chordomas anywhere in the spine were included. Extracted details included presenting symptoms, radiological findings, treatment course, follow-up, and disease progression. RESULTS: Thirty-one studies, with a total of 41 patients, were included in this review. Seventy-six percent (31/41) of patients had primary intradural tumors, whereas 24% (10/41) presented with metastasis. The most common signs and symptoms were pain (n = 27, 66%); motor deficits (n = 20, 49%); sensory deficits (n = 17, 42%); and gait disturbance (n = 10, 24%). The most common treatment for intradural chordoma was resection and postoperative radiotherapy. Sixty-six percent (19/29) of patients reported improvement or complete resolution of symptoms after surgery. The recurrence rate was 37% (10/27), and the complication rate was 25% (6/24). The median progression-free survival was 24 months (range 4-72 months). Four patient deaths were reported. The median follow-up time was 12 months (range 13 days-84 months). CONCLUSIONS: Treatment of intradural spinal chordomas primarily involves resection and radiotherapy. A significant challenge and complication in management is spinal tumor seeding after resection, with 9 studies proposing seeding as a mechanism of tumor metastasis in 11 cases. Factors such as tumor size, Ki-67 positivity, and distant metastasis may correlate with worse outcomes and demonstrate potential as prognostic indicators for intradural spinal chordomas. Further research is needed to improve understanding of this tumor and develop optimal treatment paradigms for these patients.

[Application of microchannel technique in minimally invasive resection of cervical intraspinal tumors].

OBJECTIVE: To explore the application and key points of microchannel approaches in resection of cervical intraspinal tumors. METHODS: A retrospective analysis was performed on 51 cases of cervical spinal canal tumors from February 2017 to March 2020. Among them, 5 cases were located epidural space, 6 cases were located epidural and subdural space, and 40 cases were located under the subdural extramedullary space(6 cases were located on the ventral side of the spinal cord). The maximum diameter ranged from 0.5 to 3.0 cm. The clinical manifestations included neck, shoulder or upper limb pain 43 cases, sensory disturbance (numbness) in 22 cases, and limb weakness in 8 cases. The microchannel keyhole technique was used to expose the tumor, and the tumor was resected microscopically. RESULTS: In this study, 35 patients underwent hemilaminectomy, 12 patients underwent interlaminar fenestration, 2 patients underwent medial 1/4 facetectomy on the basis of hemilaminectomy or interlaminar fenestration. Two tumors were resected through anatomy space (no bone was resected). The degree of tumor resection included total resection in 50 cases and subtotal resection in 1 case. The type of the tumor included 36 schwannomas, 12 meningiomas, 2 enterogenic cysts and 1 dermoid cyst. There was no infection and cerebrospinal fluid leakage postoperatively. Limb numbness occurred in 7 patients. The average follow-up time was 15 months (3 to 36 months). No deformity such as cervical instability or kyphosis was found. The tumor had no recurrence. CONCLUSION: The cervical spinal canal is relatively wide, cervical tumors with no more than three segments can be fully exposed by means of microchannel technology. Besides intramedullary or malignant tumors, they can be microsurgically removed. Preservation of the skeletal muscle structure of cervical spine is beneficial to recover the anatomy and function of cervical spine. The electrophysiological monitoring helps to avoid spinal cord or nerve root injury.

[Minimally invasive surgical treatment of extramedullary tumors at the level of craniovertebral junction: experience of the Burdenko Neurosurgical Center]. / Minimal'no invazivnoe khirurgicheskoe lechenie patsientov s ekstramedullyarnymi opukholyami na urovne kraniovertebral'nogo perekhoda: opyt NMITs neirokhirurgii im. akad. N.N. Burdenko.

OBJECTIVE: To describe own experience of treating patients with extramedullary tumors at the level of craniovertebral junction using minimally invasive surgical approaches. MATERIAL AND METHODS: The study included 29 patients who underwent minimally invasive microsurgical resection of extramedullary tumors at the level of craniovertebral junction. We analyzed the main clinical and surgical parameters. RESULTS: Gross total resection was achieved in most patients with high degree of safety. Two patients required redo surgery due to CSF leakage and soft tissue cyst. Mean length of hospital-stay was 7 days. VAS score of pain syndrome at discharge was 2 points and 0 points after 3 months. No significant differences in neurophysiological monitoring indicators were observed (p=0.76). CONCLUSION: Minimally invasive posterior approaches to extramedullary tumors at the level of craniovertebral junction can significantly reduce surgical trauma with equal extent of resection.

Clinical presentation and extent of resection impacts progression-free survival in spinal ependymomas.

PURPOSE: Primary treatment of spinal ependymomas involves surgical resection, however recurrence ranges between 50 and 70%. While the association of survival outcomes with lesion extent of resection (EOR) has been studied, existing analyses are limited by small samples and archaic data resulting in an inhomogeneous population. We investigated the relationship between EOR and survival outcomes, chiefly overall survival (OS) and progression-free survival (PFS), in a large contemporary cohort of spinal ependymoma patients. METHODS: Adult patients diagnosed with a spinal ependymoma from 2006 to 2021 were identified from an institutional registry. Patients undergoing primary surgical resection at our institution, ≥ 1 routine follow-up MRI, and pathologic diagnosis of ependymoma were included. Records were reviewed for demographic information, EOR, lesion characteristics, and pre-/post-operative neurologic symptoms. EOR was divided into 2 classifications: gross total resection (GTR) and subtotal resection (STR). Log-rank test was used to compare OS and PFS between patient groups. RESULTS: Sixty-nine patients satisfied inclusion criteria, with 79.7% benefitting from GTR. The population was 56.2% male with average age of 45.7 years, and median follow-up duration of 58 months. Cox multivariate model demonstrated significant improvement in PFS when a GTR was attained (p <.001). Independently ambulatory patients prior to surgery had superior PFS (p <.001) and OS (p =.05). In univariate analyses, patients with a syrinx had improved PFS (p =.03) and were more likely to benefit from GTR (p =.01). Alternatively, OS was not affected by EOR (p =.78). CONCLUSIONS: In this large, contemporary series of adult spinal ependymoma patients, we demonstrated improvements in PFS when GTR was achieved.

Capillary Hemangioma of the Spinal Cord: Case Report and Systematic Review of Literature.

BACKGROUND: Capillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with intramedullary extension rare. We present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of the literature. METHODS: Medical records and imaging data were retrospectively reviewed using the health record software EPIC (Verona, Wisconsin, USA) and the radiology management software system RIS/PACS (Radiology Information System/Picture Archiving and Communication System; QREADS). The report was written in accordance with the CARE (case reports) guidelines. We also performed a systematic review of the literature on all cases of intramedullary spinal capillary hemangiomas in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. RESULTS: We report a case of a 54-year-old man who presented with progressive paraplegia and sensory deficits in the lower extremities. Spinal magnetic resonance imaging showed an intramedullary enhancing lesion centered at T11 with associated spinal cord compression. He underwent thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological examination. Histological examination showed findings consistent with a capillary hemangioma. The literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangioma. CONCLUSIONS: Purely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature. These should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients.

Embracing Exoscope in the Arsenal of Spine Surgeons in the Low- and Middle-Income Countries.

BACKGROUND: Exoscope (EX) is a device that combines the convenience of an endoscope with the image clarity of an operating microscope (OM) to fill the void between the two. This study aims to compare the 2-dimensional EX with OM in spine surgeries and to explore its utility and feasibility in small and peripheral hospitals of low- and middle-income countries. METHODS: Eighty-two patients with intradural spinal tumors (extramedullary and intramedullary) aged more than 18 years were included between August 2021 and August 2023. Patients with other spinal pathologies were excluded. After each exoscopic surgery, the operating surgeon and assistant had to answer a questionnaire. Postoperatively, outcomes were measured as the length of hospital stay, cerebrospinal fluid leak, and number of reoperations. RESULTS: Thirty-seven patients were included in the OM group and 45 patients were included in the EX group. In 62%-67% of cases, the overall image quality of the EX was comparable to OM and in 29%-38% of cases, it was superior to the OM. The preparation and installation of the EX were much easier and better than the OM in 93%-100% of the cases. Maneuvering surgical instruments and workflow in the operating theater was much more convenient in the EX group (95%-100% of the cases). Ergonomics was far better in the EX group than in the OM group. CONCLUSIONS: Spine surgeons can embrace the benefits of EX and increase their range of surgeries to be performed at the small operation theater setup in low-middle income developing countries.

Clinical Features and Surgical Management of Intramedullary Schwannoma of the Spinal Cord.

BACKGROUND: Intramedullary schwannoma is a relatively rare tumor with only a few literature reports. This study was aimed to report the clinical characteristics of intramedullary schwannoma and discuss imaging findings and treatment strategies. METHODS: The inclusion criterion was consecutive patients with intramedullary schwannomas who were surgically treated in our institution between 2017 and 2022. Data included clinical characteristics, radiologic features, surgical management, and prognosis. Clinical and follow-up details of all cases were collected and reviewed. RESULTS: This study included 3 male and 8 female patients. The mean age was 45 years (range 26-77 years). Cervical spine (4 cases, 36.4%), thoracic spine (4 cases, 36.4%), and lumbosacral spine (3 cases, 27.3%) involvement was found. Weakness, numbness and pain of limbs were the main symptoms at administration. Preoperative magnetic resonance imaging demonstrated lesion with spinal cord medullary invasion and well demarcated margins. The postoperative histologic examination showed benign lesions and confirmed the schwannoma. CONCLUSIONS: This article presented a series of 11 cases of intramedullary schwannoma with sharp margins and well-enhanced features. Prognosis and functional recovery were good after gross total resection.

Survival and neurological outcomes following management of intramedullary spinal metastasis patients: a case series with comprehensive review of the literature.

Intramedullary spinal cord metastasis (ISCM), though rare, represents a potentially debilitating manifestation of systemic cancer. With emerging advances in cancer care, ISCMs are increasingly being encountered in clinical practice. Herein, we describe one of the larger retrospective single institutional case series on ISCMs, analyze survival and treatment outcomes, and review the literature. All surgically evaluated ISCMs at our institution between 2005 and 2023 were retrospectively reviewed. Demographics, tumor features, treatment, and clinical outcome characteristics were collected. Neurological function was quantified via the Frankel grade and the McCormick score (MCS). The pre- and post-operative Karnofsky performance scores (KPS) were used to assess functional status. Descriptive statistics, univariate analysis, log-rank test, and the Kaplan-Meier survival analysis were performed. A total of 9 patients were included (median age 67 years (range, 26-71); 6 were male). Thoracic and cervical spinal segments were most affected (4 patients each). Six patients (75%) underwent surgical management (1 biopsy and 5 resections), and 3 cases underwent chemoradiation only. Post-operatively, 2 patients had an improvement in their neurological exam with one patient becoming ambulatory after surgery; three patients maintained their neurological exam, and 1 had a decline. There was no statistically significant difference in the pre- and post-operative MCS and median KPS scores in surgically treated patients. Median OS after ISCM diagnosis was 7 months. Absence of brain metastasis, tumor histology (renal and melanoma), cervical/thoracic location, and post-op KPS ≥ 70 showed a trend toward improved overall survival. The incidence of ISCM is increasing, and earlier diagnosis and treatment are considered key for the preservation of neurological function. When patient characteristics are favorable, surgical resection of ISCM can be considered in patients with rapidly progressive neurological deficits. Surgical treatment was not associated with an improvement in overall survival in patients with ISCMs.

Intraoperative Neurophysiologic Monitoring and Mapping During Surgery on Intramedullary Spinal Cord Tumors in Children and Adolescents.

SUMMARY: Surgical resection of intramedullary spinal cord tumors carries significant risks of neurologic deficits, especially in cases of infiltrative tumors. In pediatric patients, this type of surgery may be associated with a high risk of poor neurologic outcome. Intraoperative neurophysiologic monitoring has been adopted as part of the clinical routine by many centers as a useful adjunct for intraoperative assessment of neurologic integrity. To what extent intraoperative neurophysiologic mapping strategies may further support intraoperative decision-making is still a matter of debate. Here, we report on a small cohort of five pediatric patients in whom mapping with the double-train paradigm was used to identify the dorsal column and corticospinal tract and to guide the surgical resection. We also discuss the possible benefits and challenges regarding the available literature.

Intramedullary spinal cord tumors in pediatric patients presenting later with brain lesions: case series and systematic review of the literature.

PURPOSE: Intramedullary spinal cord tumors are an uncommon pathology in adults and children. Most descriptive studies of intramedullary spinal cord tumors have not focused on a possible association with future brain lesions. To the best of our knowledge, few reports describe this potential relationship. This is one of the most extensive case series of secondary brain lesions of intramedullary spinal cord tumors in the pediatric population. METHODS: Retrospective chart review was performed on pediatric patients (21 years old and younger) who underwent resection of an intramedullary spinal cord tumor at two tertiary care hospitals from 2001 to 2020. Patients previously treated or diagnosed with spinal cord tumor, and subsequent development of intracranial manifestation of the same or different tumor, were included. Data regarding epidemiology, surgical intervention, and clinical and follow-up course were gathered. Data analysis was performed according to a standardized clinical protocol with a literature review. RESULT: More than 500 patients underwent intradural spinal tumor resection surgeries at participating hospitals from 2001 to 2020. After excluding adult patients (older than 21 years old) and those with extramedullary lesions, 103 pediatric patients were identified who underwent resection of an intramedullary spinal cord tumor. Four underwent resection of an intermedullary tumor and later in their follow-up course developed a secondary intracranial neoplasm. In every case, the secondary neoplasm had the same pathology as the intramedullary tumor. Three of the patients had tumors at the cervico-thoracic junction, and one patient had a high cervical tumor. These patients had a negative primary workup for any metastatic disease at the time of the presentation or diagnosis. Complete and near complete resection was performed in three patients and subtotal in one patient. CONCLUSION: Secondary brain tumors disseminated after initial spinal cord tumor are extremely rare. This study aims to allow specialists to better understand these pathologies and treat these rare tumors with more certainty and better expectations of unusual associated lesions and conditions.

Spinal intramedullary uterine carcinosarcoma metastasis.

Intramedullary spinal cord metastases (ISCM) are a rare and challenging manifestation of metastatic cancer that have devastating impacts on the individual's neurological function, survival expectancy and overall quality of life. Given the rarity and poor prognosis, there is a lack of consensus in management. Uterine carcinosarcoma itself is a rare cancer, accounting for less than 3% of all uterine cancers. It carries a poor prognosis, with only one-third of patients surviving beyond 5 years. There are no previous reports of uterine carcinosarcoma metastases to the spinal cord. Here, we present the case of a woman in her late 70s with a uterine carcinosarcoma intramedullary metastasis that was refractory to radiotherapy treatment and responded favourably to surgical debulking.

Far lateral approach for dumbbell-shaped C1 schwannomas: how I do it.

BACKGROUND: Dumbbell-shaped C1 schwannomas are rare lesions that involve both intra- and extradural compartments. Because of the intimate relationships these lesions develop with the third and fourth segments of the vertebral artery, surgical removal of these lesions remains a challenge. METHOD: We describe the key steps of the far lateral approach for dumbbell-shaped C1 schwannomas with a video illustration. The surgical anatomy is described along with the techniques for protecting the vertebral artery. CONCLUSION: Dumbbell-shaped C1 schwannomas can be safely removed by using the far lateral approach, surgical anatomy expertise, and intraoperative microvascular Doppler.

[Laser fluorescence spectroscopy and navigation in surgical treatment of spinal tumors: a systematic review]. / Primenenie lazernoi flyuorestsentnoi spektroskopii i navigatsii pri khirurgicheskom lechenii opukholei spinnogo mozga i pozvonochnika: sistematicheskii obzor literatury.

The main problem in microsurgical resection of spinal cord tumors is excessive surgical aggression. The last one often leads to unsatisfactory clinical and neurological outcomes. Laser fluorescence spectroscopy is a modern neurosurgical approach to distinguish tumor boundaries even if standard visible fluorescence techniques are ineffective. OBJECTIVE: To evaluate the effectiveness of laser fluorescence spectroscopy alone or in combination with visual 5-ALA fluorescence for improvement of safety and quality of resection, as well as intraoperative diagnosis. MATERIAL AND METHODS: Searching for literature data was carried out in accordance with the PRISMA recommendations. The authors reviewed articles independently of each other. These data were systematized. RESULTS: Laser fluorescence spectroscopy is valuable to identify fragments of intramedullary ependyomas in 86% of cases, while visual fluorescence only in 81% of cases. Advisability of this technique for low-grade astrocytomas is still unclear and requires further study. Its effectiveness is 87.5% for extramedullary meningiomas. Neuromas do not accumulate 5-ALA. In addition, this method can be used to determine the boundaries of intradural metastatic lesions. CONCLUSION: 5-ALA fluorescence is a safe and useful intraoperative method for identifying tumor tissue and resection margins in patients with intramedullary or infiltrative extramedullary spinal cord tumors. Visual fluorescence combined with laser spectroscopy is a perspective method for intraoperative visualization of tumor remnants. This approach can improve safety and postoperative outcomes while maintaining resection quality.

Complex lumbosacral spinal cord lipomas: A longitudinal study on outcomes from a Singapore children's hospital.

BACKGROUND: Total/near-total resection (TR/NTR) of complex lumbosacral lipomas (CSL) is reported to be associated with better long-term functional outcomes and lower symptomatic re-tethering rates. We report our institutional experience for CSL resection in affected children. METHODS: This is a single-institution, retrospective study. Inclusion criteria consist of patients with CSL with dorsal, transitional and chaotic lipomas based on Pang et al's classification. The study population is divided into 2 groups: asymptomatic patients with a normal preoperative workup referred to as 'prophylactic intent' and 'therapeutic intent' for those with pre-existing neuro-urological symptoms. Primary aims are to review factors that affect post-operative clean intermittent catheterization (CIC), functional outcomes based on Necker functional score (NFS), and re-tethering rates. RESULTS: 122 patients were included from 2000 to 2021. There were 32 dorsal lipomas (26.2 %), 74 transitional lipomas (60.7 %), and 16 chaotic lipomas (13.1 %). 82 % patients achieved TR/NTR. Favourable NFS at 1-year was 48.2 %. The re-tethering rate was 6.6 %. After multivariable analysis, post-operative CIC was associated with median age at surgery (p = 0.026), lipoma type (p = 0.029), conus height (p = 0.048) and prophylactic intent (p < 0.001). Next, extent of lipoma resection (p = 0.012) and the post-operative CSF leak (p = 0.004) were associated with re-tethering. Favourable NFS was associated with lipoma type (p = 0.047) and prophylactic intent surgery (p < 0.001). CONCLUSIONS: Our experience shows that TR/NTR for CSL is a feasible option to prevent functional deterioration and re-tethering. Efforts are needed to work on factors associated with post-operative CIC.

Digital 3D Exoscope is Safe and Effective in Surgery for Intradural Extramedullary Tumors: A Comparative Series.

BACKGROUND: Digital 3D exoscopes have been shown to be comparably safe and effective as surgical microscopes in complex microneurosurgical procedures. However, the results of exoscopic spinal tumor surgeries are scarce. The purpose of this study is to compare results of a transition from microscope to exoscope in surgeries for spinal intradural extramedullary tumors. METHODS: We included all consecutive patients with intradural extramedullary spinal tumors operated on by the senior author during January 2016 to October 2023. The 3D exoscope was used in the latter half of the series from November 2020. We evaluated pre- and postoperative clinical findings, imaging studies, intra- and postoperative events, and analyzed surgical videos from the operations retrospectively. RESULTS: We operated 35 patients (exoscope n = 19, microscope n = 16) for intradural extramedullary tumors (meningioma n = 18, schwannoma n = 12, other n = 5). Tumors in the cervical and thoracic spine were more common than in the lumbar region. The duration of surgery was slightly longer (median 220 vs. 185 minutes) in the exoscope group. However, the rate of gross total resection of the tumor was higher (81% vs. 67%) and the tumors more often located anteriorly to the spinal cord (42% vs. 13%) in the exoscope group. No major complications (i.e., permanent motor deficit or postoperative hematoma) occurred in either group. We saw postoperative gait improvement in 81% and 85% of the patients with preoperative deterioration of gait after exoscopic and microscopic surgeries, respectively. CONCLUSIONS: This study demonstrates that exoscope-assisted surgery for spinal intradural extramedullary tumors is comparable in safety and effectiveness to traditional microscopic surgery.

Intraoperative High-Resolution Color-Coded Ultrasound to Identify the Posterior Median Sulcus for Midline Myelotomy in Intramedullary Spinal Cord Surgery.

A 14-year-old boy presented with a 2-year history of slowly increasing weakness and atrophy in the right forearm and leg. Magnetic resonance imaging (MRI) revealed an intramedullary diffusely infiltrating lateralized tumor at C3-7. An extended biopsy was planned. After laminotomy and durotomy, the swollen spinal cord was noted to be rotated by 45° with the right dorsal root entry zone being in the midline. A 15 MHz linear ultrasound probe was used to identify the midline by visualizing the dorsal median sulcal vein within the midline raphe. A myelotomy was made in that zone without deterioration of somatosensory evoked potentials (SEPs) and an extended biopsy was performed. Histological examination revealed a pilocytic astrocytoma. Modern intraoperative high-resolution color-coded ultrasound enables the identification of the midline in intramedullary spinal cord lesions even when the spinal cord anatomy is distorted.

Giant Myxopapillary Ependymoma with Multi-Site Neural Axis Metastases: A Rare Case with Suboptimal Outcome.

BACKGROUND Myxopapillary ependymoma is a rare type of slow-growing tumor that mainly occurs in the spinal cord, particularly in the region of the conus medullaris and the cauda equina. It originates from the ependymal glial cells found in the filum terminale. CASE REPORT We present a clinical case of a 44-year-old male patient who presented with symptoms of non-specific pain in the lower back persisting for the past 2 years. He did not report any specific neurological deficits or radicular symptoms. Unenhanced MRI of the lumbar spine showed a giant intradural, extramedullary, heterogenous, expansive tumor at the level L1-S4 with erosion of the sacral bone and invasion of presacral tissue. Based on its characteristic localization and growth pattern, suspicion arose for myxopapillary ependymoma. Biopsy confirmed the initial diagnosis. Partial resection of the tumor with laminectomy and laminoplasty was deemed necessary. Preoperative neural axis MRI showed contrast-enhancing lesions in the cerebellum and the cervical and thoracic spine; therefore, adjuvant radiation therapy was administered. Following the surgery, the patient experienced intermittent episodes of neurological deficits and required physiotherapy. Control MRI a year after the operation showed tumor growth and more metastases along the neural axis. CONCLUSIONS Complete surgical excision of the tumor is the preferred treatment approach, but there is a risk of recurrence even after total excision, so radiotherapy is recommended to minimize the risk of recurrence. Prior to surgery, it is essential to conduct MRI/PET/CT of the head and spine to assess the possibility of metastases.

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.