Primary extraskeletal intradural Ewing sarcoma with acute hemorrhage: a case report and review of the literature.

BACKGROUND: Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management. CASE PRESENTATION: We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted. CONCLUSIONS: Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.

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.

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.

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.

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.

Spinal Cord Neoplasms.

OBJECTIVE: This article discusses the diagnostic approach to patients with suspected neoplasms of the spinal cord and reviews the most common primary and metastatic spinal neoplasms and their presentations. LATEST DEVELOPMENTS: Neoplasms of the spinal cord are rare entities that can involve the spinal cord parenchyma, the dura and leptomeninges, or the extradural space. The most common intramedullary spinal cord neoplasms are primary spinal cord tumors, including ependymomas, pilocytic astrocytomas, and diffuse midline gliomas. The most common primary neoplasms of the spine are intradural extramedullary spinal meningiomas, whereas primary neoplasms of the leptomeninges are rare. Advances in molecular characterization of spinal cord tumors and recent clinical trials of these rare entities are expanding the repertoire of systemic therapy options for primary spinal cord neoplasms. Metastases to the spine most often affect the extradural space. Metastatic epidural spinal cord compression is a neurologic emergency that requires a rapid, multidisciplinary response to preserve neurologic function. ESSENTIAL POINTS: Neurologists should understand the diagnostic approach to neoplasms of the spinal cord. Knowledge of the most common spinal cord neoplasms will allow for appropriate management and optimal patient care.

[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.

Intraoperative mixed-reality spinal neuronavigation system: a novel navigation technique for spinal intradural pathologies.

OBJECTIVE: The objective of this study was to assess the intraoperative accuracy and feasibility of 3D-printed marker-based mixed-reality neurosurgical navigation for spinal intradural pathologies. METHODS: The authors produced 3D segmentations of spinal intradural tumors with neighboring structures by using combined CT and MRI, and preoperative registration of pathology and markers was successfully performed. A patient-specific, surgeon-facilitated application for mobile devices was built, and a mixed-reality light detection and ranging (LIDAR) camera on a mobile device was employed for cost-effective, high-accuracy spinal neuronavigation. RESULTS: Mobile device LIDAR cameras can successfully overlay images of virtual tumor segmentations according to the position of a 3D-printed marker. The surgeon can visualize and manipulate 3D segmentations of the pathology intraoperatively in 3D. CONCLUSIONS: A 3D-printed marker-based mixed-reality spinal neuronavigation technique was performed in spinal intradural pathology procedures and has potential to be clinically feasible and easy to use for surgeons, as well as being time saving, cost-effective, and highly precise for spinal surgical procedures.

Resection of an Intradural Intramedullary C7-T1 Tumor: Technical Nuances and Complication Management.

Primary spinal cord tumors are relatively rare, comprising approximately 4%-16% of all tumors originating from the central nervous system. These tumors are anatomically separable into 2 broad categories: intradural intramedullary and intradural extramedullary. Intramedullary tumors are composed predominantly of gliomas (infiltrative astrocytoma) and ependymomas.1-4 The primary treatment approach for these tumors is surgical resection, aiming to preserve neurologic function.5-9 In Video 1, the authors showcase a step-by-step approach for microsurgical resection of a primary spinal ependymoma, with emphasis on microsurgical technique and utility of adjunct equipment, such as intraoperative ultrasound and neuromonitoring.10,11 The patient consented to the procedure.

Upstream Effects: Intraoperative Migration of Intradural Extramedullary Tumor After Non-Adjacent Level Stenosis Decompression.

A 74-year-old man with back pain, foot numbness, and hip/thigh radiculopathy was found to have an L1-L2 intradural extramedullary neoplasm and severe L4-L5 stenosis. L4-L5 minimally invasive laminectomy for decompression and concomitant L1-L2 minimally invasive laminectomy for tumor resection were planned. L4-L5 laminectomy was completed first followed by the L1-L2 laminectomy. On extensive intradural exploration at L1-L2, no neoplasm was found. Immediate postoperative imaging showed that the intradural extramedullary tumor had migrated caudally by nearly a complete spinal level, presumably due to changes in cerebrospinal fluid pressure and resultant shift in intradural contents after the L4-L5 laminectomy. Successful resection of the intradural extramedullary tumor was performed, with improvement in the patient's symptoms.

Usefulness of magnetic resonance imaging characteristics in discriminating H3 K27M-mutant gliomas from wildtype gliomas in spinal cord.

AIM: The aim of this study was to determine the usefulness of magnetic resonance imaging (MRI) characteristics in discriminating H3 K27M-mutant gliomas from wildtype gliomas in the spinal cord. MATERIALS AND METHODS: Fifty-eight patients with spinal cord gliomas were enrolled in this study. The H3 K27 gene status was identified by Sanger sequencing or immunohistochemistry test of resection tumor specimens. The MR imaging characteristics were evaluated and compared between H3 K27M-mutant and wildtype gliomas using the χ2 test and the Mann-Whitney U test. RESULTS: Of 58 recruited patients, 23 (39.7%) were diagnosed with H3 K27M-mutant glioma. The H3 K27M-mutant gliomas were found to more likely occur in men compared with wildtype gliomas (87.0% vs. 42.9%, p = 0.001). On T2-weighted MR images, the signal-to-noise ratio (SNR) of H3 K27M-mutant gliomas was significantly lower than that of wildtype gliomas (103.9 ± 72.0 vs. 168.9 ± 86.8, p < 0.001). Of 35 wildtype tumors, 60% showed well-defined margin but this feature was not found in all mutant tumors (p < 0.001). The SNR of tumors on contrast-enhanced T1-weighted images of the H3 K27M-mutant gliomas was significantly lower than that of wildtype gliomas (187.7 ± 160.4 vs. 295.1 ± 207.8, p = 0.006). Receiver operating-characteristic analysis revealed that area under curve (AUC) of combination of 1/SNR on T2-weighted images, 1/SNR on contrast-enhanced T1-weighted images, ill-defined margin, and sex reached 0.937 (95% CI, 0.873-1.000) in discriminating H3 K27M-mutant gliomas. CONCLUSIONS: The MR imaging characteristics are valuable in discriminating H3 K27M-mutant from wildtype gliomas in the spinal cord and the combination of these imaging features with sex had a high strength in this discrimination.

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 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.

Comparison of machine learning algorithms for the classification of spinal cord tumor.

Spinal cord Tumor has been characterized as a heterogeneous disease consisting of many different subtypes. The early diagnosis and prognosis of a cancer type have become a necessity in cancer research, as it can facilitate the subsequent clinical management of patients. The importance of classifying cancer patients into Bening or malignant has led many re- search teams, from the biomedical and the bioinformatics field, to study the application of machine learning (ML) methods. Therefore, these techniques have been utilized as an aim to model the progression and treatment of cancerous conditions. In addition, the ability of ML tools to detect key features from complex datasets reveals their importance. A variety of these techniques, Logistic regression, Support Vector Machines (SVMs), Decision Trees (DTs), Random forest classifier(RFs) have been widely applied in cancer research for the development of predictive models, resulting in effective and accurate decision making. Even though it is evident that the use of ML methods can improve our understanding of cancer progression, an appropriate level of validation is needed in order for these methods to be considered in the everyday clinical practice. In this work, we have discussed a predictive model based on various supervised ML techniques.

A feline spinal dermoid cyst treated with surgical intervention.

A 5-year-old neutered female mixed cat presented with reduced activity and ataxia of the hind limbs. Computed tomography and magnetic resonance imaging revealed an extradural mass compressing the spinal cord on the dorsal aspects from the 7th to 8th thoracic vertebra. Dorsal laminectomy was performed on the 7-8th thoracic vertebra and the cyst was totally removed, giving full resolution of the clinical signs. The cyst was diagnosed as a dermoid cyst. To our knowledge, this is the first report of feline dermoid cyst compressing the spinal cord that was diagnosed antemortem. The prognosis is favorable when the cyst is completely resected.