Characterization of spinal hemangioblastomas in patients with and without von Hippel-Lindau, and YAP expression.

INTRODUCTION: Hemangioblastoma (HB) is a benign tumor of the central nervous system, associated with von Hippel-Lindau disease (VHL), or sporadic. The aim of this study was to compare and examine the clinical-pathological profile of patients with spinal hemangioblastoma and YAP expression. METHODS: A retrospective, descriptive, comparative study. All patients who underwent surgery for spinal HB between 2016 and 2023 were included. Clinical and radiological data were collected and analyzed. An immunohistochemistry panel including NeuN, neurofilaments (NF), and YAP-1, was performed. RESULTS: Nine patients were studied, six women and three men. Four patients had previously diagnosed VHL. The tumor location included: four cervical (44.44%), two thoracic (22.22%), two pontine with cervical extension (22.22%) and one patient with two lesions, one cervical and one thoracic (11.11%). Non-significant clinical differences were identified between VHL and sporadic patients. Imaging evidenced seven extramedullary and three intramedullary tumors. Histologically, intra-tumoral and perivascular axonal tracts were observed in all cases. One third of the tumors (two with VHL and one sporadic) presented extramedullary hematopoiesis. Seven cases (77.8%) expressed nuclear YAP (three with VHL and four sporadic HBs). The surgical outcome was good and only one patient with VHL undergoing subtotal resection had recurrence. CONCLUSIONS: Spinal HBs can be associated with VHL or be sporadic. To the best of our knowledge, this is the first study to describe YAP expression in HB. It is important to investigate the involvement of the Hippo pathway in HBs as a possible therapeutic target.

Holocord pilocytic astrocytoma in a young woman with intracranial extension: case report and review of the MRI characteristics.

INTRODUCTION: Pilocytic astrocytoma is a low-grade glioma more frequently seen in patients <20. It is pretty uncommon in the spinal cord. Rarely, astrocytoma may involve the most or total length of the spinal cord; in that case, they are called "holo-cord astrocytoma." In this case report, we are reporting the third holo-cord pilocytic astrocytoma in an adult patient and the first with an extension to the Magendie foramen. CASE PRESENTATION: We presented a 24-year-old woman with complaints of progressively worsening neck and back pain since one year ago. The patient's MRI showed a very large intradural and intramedullary cystic lesion with a solid component within the spinal cord extending from the medulla to the conus medullaris. Partial resection of the solid part of the cervical portion of the tumor was performed. Histopathological evaluation of the resected tumor segments was compatible with grade I pilocytic astrocytoma. After one year of follow-up, neck and back pain has reduced, and neurological functions have improved. CONCLUSION: Spinal cord pilocytic astrocytoma may present as a holo-cord tumor and can rarely extend to the intracranial fossa. Although this tumor does not arise from the central canal, in this case, it was extended through the Magendie foramen. Symptoms could be subtle despite extensive cord involvement. On MRI, this tumor presents as an intramedullary holo-cord cystic lesion intermixed with a solid component with a variable enhancement of the solid component.

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.

Malignant melanotic schwannoma of the cervical spinal cord: a case report.

Spinal cord malignant melanotic schwannoma (MMNST) is a rare central nervous system tumor that originates from the spinal cord or spinal myelin sheath cells and can produce melanin. This type of tumor is usually highly aggressive and malignant, with a poor prognosis. The clinical manifestations of spinal cord MMNST are mainly pain, paresthesia, muscle weakness, muscle atrophy, etc., and symptoms of spinal cord compression, such as intestinal and bladder dysfunction, paraplegia, etc. Early detection of tumor lesions can facilitate tumor removal, improve patients' quality of life, and prolong patients' survival. In this case report, a 27-year-old young woman was diagnosed with MMNST of the cervical spinal cord due to weakness of her limbs in our hospital, and underwent surgical resection. The patient's limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for "right upper limb pain for 3 days" and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was "hemosiderosis". The patient's limbs returned to normal after surgery. It is worth mentioning that the patient visited our hospital 7 months ago for "right upper limb pain for 3 days" and was diagnosed with a cervical spine space-occupying lesion at the same position this time, but the pathology report was "hemosiderosis". This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor. This case report aims to raise awareness of the problem of spinal cord MMNST and contribute to greater knowledge of this rare tumor.

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.

Patient-reported outcomes in Primary Spinal Intradural Tumours: a systematic review.

STUDY DESIGN: Systematic review. OBJECTIVES: Primary Spinal Intradural Tumours (PSITs) are rare pathologies that can significantly impact quality of life. This study aimed to review patient reported outcomes (PROs) in PSITs. METHODS: A systematic search of Pubmed and Embase was performed to identify studies measuring PROs in adults with PSITs. PRO results were categorised as relating to Global, Physical, Social, or Mental health. Outcomes were summarised descriptively. RESULTS: Following review of 2382 records, 11 studies were eligible for inclusion (737 patients). All studies assessed surgically treated patients. Schwannoma was the commonest pathology (n = 190). 7 studies measured PROs before and after surgery, the remainder assessed only post-operatively. For eight studies, PROs were obtained within 12 months of treatment. 21 PRO measurement tools were used across included studies, of which Euro-Qol-5D (n = 8) and the pain visual/numerical analogue scale (n = 5) were utilised most frequently. Although overall QoL is lower than healthy controls in PSITs, improvements following surgery were found in Extramedullary tumours (EMT) in overall physical, social, and mental health. Similar improvements were not significant across studies of Intramedullary tumours (IMT). Overall QoL and symptom burden was higher in IMT patients than in brain tumour patients. No studies evaluated the effect of chemotherapy or radiotherapy. CONCLUSION: Patients with PSITs suffer impaired PROs before and after surgery. This is particularly true for IMTs. PRO reporting in PSITs is hindered by a heterogeneity of reporting and varied measurement tools. This calls for the establishment of a standard set of PROs as well as the use of registries.

Predictive Factors of Long-Term Neurologic Outcome and Progression-Free Survival in Intramedullary Spinal Cord Tumors: A 10-year Single-Center Cohort Study and Review of the Literature.

BACKGROUND: Intramedullary spinal cord tumors (IMSCTs) are a rare subgroup of neoplasms, encompassing both benign, slow-growing masses, and malignant lesions; radical surgical excision represents the cornerstone of treatment for such pathologies regardless of histopathology, which, on the other hand, is a known predictor of survival and neurologic outcome postsurgery. The present study aims to investigate the relevance of other factors in predicting survival and long-term functional outcomes. METHODS: We conducted a review of current literature on functional outcomes of IMSCTs, as well as a 10-years prospective analysis of a wide cohort of patients with diagnosis of IMSCTs who underwent surgical resection at our institution. RESULTS: Our series encompasses 60 patients with IMSCTS, among which 36 ependymomas, 6 cavernous angiomas, 5 hemangioblastomas, 6 WHO Grade I-IV astrocytomas, 3 intramedullary spinal metastases and 4 miscellaneous tumors. GTR was achieved in 76,67% of patients, with high preoperative McCormick grade, syringomyelia and changes at neurophysiologic monitoring being the strongest predictors at multivariate analysis (P = 0.0027, P = 0.0017 and P = 0.001 respectively). CONCLUSIONS: Consistently with literature, preoperative neurologic function is the most important factor predicting long-term functional outcome (0.17, CI 0.069-0.57 with P = 0.0018), advocating for early surgery in the management of IMSCTs, whereas late complications such as myelopathy and neuropathic pain were present regardless of preoperative function.

Longitudinal Pulse-Synchronous Bouncing During Catheter Angiography-A Phenomenon Specific to Spinal Hemangioblastomas.

BACKGROUND: Spinal hemangioblastomas are often evaluated with catheter angiography for both workup and treatment planning. We report a unique longitudinal pulse-synchronous bouncing phenomenon observed during their angiographic evaluation and consider the association of pulse-synchronous bouncing with syringomyelia, another pathologic feature associated with hemangioblastomas. METHODS: Preoperative spinal angiograms and associated magnetic resonance imagings (MRIs) obtained over a 16-year period at a single institution were retrospectively evaluated. Magnetic resonance imaging (MRI) parameters included lesion and syrinx location and size. Angiograms were evaluated for bouncing phenomena. Student's t-test and Chi square test compared characteristics between groups. Linear regression analyses evaluated maximum amplitude of dynamic motion and any associated syrinx. RESULTS: Nineteen hemangioblastoma patients had preoperative angiograms available for review. Eight exhibited bouncing behavior. Between the dynamic and nondynamic cohorts, there was no difference in presence or volume of syrinxes. Lesions in the dynamic cohort trended towards a cervical location (75% vs. 36.3%, P = 0.10). No significant correlation was found between bouncing amplitude and syrinx size (R2 = 0.023). Dural contact may be related to this dynamic behavior since other high-flow lesions like AVMs do not demonstrate this phenomenon, and AVMs are pial-based and more likely to contact stationary dura. Here, there were fewer lesions abutting the thecal sac in the dynamic cohort (50% vs. 81.8%, P = 0.14). CONCLUSIONS: Though no significant relationship was established between this bouncing behavior and syrinx formation, noted trends included a greater range of motion for cervical lesions and limited motion in tumors abutting the thecal sac.

Single-Center Experience of Resection of 120 Cases of Intradural Spinal Tumors.

BACKGROUND: Our study presents a single-center experience of resection of intradural spinal tumors either with or without using intraoperative computed tomography-based registration and microscope-based augmented reality (AR). Microscope-based AR was recently described for improved orientation in the operative field in spine surgery, using superimposed images of segmented structures of interest in a two-dimensional or three-dimensional mode. METHODS: All patients who underwent surgery for resection of intradural spinal tumors at our department were retrospectively included in the study. Clinical outcomes in terms of postoperative neurologic deficits and complications were evaluated, as well as neuroradiologic outcomes for tumor remnants and recurrence. RESULTS: 112 patients (57 female, 55 male; median age 55.8 ± 17.8 years) who underwent 120 surgeries for resection of intradural spinal tumors with the use of intraoperative neuromonitoring were included in the study, with a median follow-up of 39 ± 34.4 months. Nine patients died during the follow-up for reasons unrelated to surgery. The most common tumors were meningioma (n = 41), schwannoma (n = 37), myopapillary ependymomas (n = 12), ependymomas (n = 10), and others (20). Tumors were in the thoracic spine (n = 46), lumbar spine (n = 39), cervical spine (n = 32), lumbosacral spine (n = 1), thoracic and lumbar spine (n = 1), and 1 tumor in the cervical, thoracic, and lumbar spine. Four biopsies were performed, 10 partial resections, 13 subtotal resections, and 93 gross total resections. Laminectomy was the common approach. In 79 cases, patients experienced neurologic deficits before surgery, with ataxia and paraparesis as the most common ones. After surgery, 67 patients were unchanged, 49 improved and 4 worsened. Operative time, extent of resection, clinical outcome, and complication rate did not differ between the AR and non-AR groups. However, the use of AR improved orientation in the operative field by identification of important neurovascular structures. CONCLUSIONS: High rates of gross total resection with favorable neurologic outcomes in most patients as well as low recurrence rates with comparable complication rates were noted in our single-center experience. AR improved intraoperative orientation and increased surgeons' comfort by enabling early identification of important anatomic structures; however, clinical and radiologic outcomes did not differ, when AR was not used.

[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.

Resection of intradural spinal lesions with concomitant instrumented fusion in children: a systematic review and representative cases.

OBJECTIVE: More than one-third of pediatric patients who undergo resection of intradural spine lesions develop progressive postoperative deformity, with as many as half of these patients subsequently requiring surgical fusion. Intradural spinal procedures with simultaneous instrumented fusion in children, however, are infrequently performed. Moreover, the rationale for patient selection, outcomes, and safety of this single-stage surgery in children has not been systematically investigated. In this study, the authors review the practice of simultaneous intradural spinal resection and instrumented fusion in pediatric patients and provide two representative case examples from their institution. METHODS: The authors searched the PubMed and Embase databases and performed a systematic review following the PRISMA protocol. Original articles of pediatric patients (age ≤ 18 years) who underwent intradural spine surgery, regardless of pathology, with concomitant instrumented fusion and reported outcomes were included. An institutional database of all spinal operations with instrumented fusion performed in patients aged ≤ 18 years over a 3-year period was screened to identify those who underwent intradural spine surgery with concomitant fusion. RESULTS: Nine patients (median age 12 years) from 6 studies who underwent intradural lesion resection and concomitant fusion met inclusion criteria. Among all 11 patients included, primary rationales for concomitant fusion were extensive bone removal (i.e., corpectomy or total facetectomy, 73%), concerns for deformity in the setting of multilevel laminectomy/laminoplasty (18%), and severe baseline deformity (9%). The most represented pathology was neurenteric cyst (55%) followed by schwannoma (18%). Myxopapillary ependymoma, granular cell tumor, and pilocytic astrocytoma each were seen in 1 case. Seven patients (64%) underwent an anterior-approach corpectomy, tumor resection, and fusion, while the remaining 4 patients (36%) underwent a posterior approach. All patients with at least 1 year of follow-up cases achieved bony fusion. CSF leak and new-onset neurological deficit each occurred in 9% (1/11). CONCLUSIONS: The rationales for performing single-stage intradural resection and fusion in pediatric patients in studies to date include the presence of severe baseline deformity, large extent of bone resection, and multilevel laminectomy/laminoplasty across cervicothoracic or thoracolumbar junctions. As current literature involving this cohort is limited, more data are needed to determine when concomitant fusion in intradural resections is appropriate in pediatric patients and whether its routine implementation is safe or beneficial.

Spinal Intramedullary Meningiomas: A Systematic Review with a Case Illustration.

BACKGROUND: Intramedullary meningiomas are an exceptionally rare subtype of spinal tumors, accounting for only 5% of primary spinal neoplasms. Given their scarcity and unique characteristics, understanding optimal management approaches is crucial for improved clinical decision-making. This systematic review aims to consolidate existing literature and present a detailed case illustration to enhance understanding of this uncommon spinal tumor entity. METHODS: A systematic search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Eligible studies included case reports, case series, cohort studies, reviews, and meta-analyses. Data extraction and synthesis focused on demographic characteristics, tumor location, clinical presentation, imaging findings, surgical interventions, histopathological features, and outcomes. RESULTS: A total of 15 high-quality scientific articles were included in the systematic review, providing insights into various aspects of intramedullary meningiomas. Demographic analysis revealed a broad age distribution with an equal gender distribution among affected patients. Common clinical presentations included difficulty walking, sensory disturbances, spastic paraparesis, and urinary incontinence. Neuroimaging findings demonstrated heterogeneous signal intensity variations on T1- and T2-weighted images, with variable enhancement patterns on gadolinium-enhanced images. Surgical interventions, predominantly total resection, resulted in favorable postoperative outcomes in most cases. CONCLUSIONS: Intramedullary meningiomas pose diagnostic and therapeutic challenges due to their rarity and unique characteristics. Tailored surgical approaches, incorporating techniques such as intraoperative neurophysiological monitoring and fluorescence-aided resection, are crucial for minimizing neurological deficits and optimizing patient outcomes. Despite their infrequency, recognizing intramedullary meningiomas in the differential diagnosis of spinal tumors is essential for prompt diagnosis and timely intervention, ultimately improving patient prognosis.

Imaging of Adult Malignant Soft Tissue Tumors of the Spinal Canal: A Guide for Spine Surgeons.

BACKGROUND: Malignant soft tissue spinal canal tumors compromise 20% of all spinal neoplasms. They may be primary or metastatic lesions, originating from a diverse range of tissues within and surrounding the spinal canal. These masses can present as diverse emergencies such as secondary cauda equina syndrome, vascular compromise, or syringomyelia. Interpretation of malignant soft tissue spinal canal tumors imaging is an essential for non-radiologists in the setting of emergencies. This task is intricate due to a great radiologic pattern overlap among entities. METHODS: We present a step-by-step strategy that can guide nonradiologists identify a likely malignant soft tissue lesion in the spinal canal based on imaging features, as well as a review of the radiologic features of malignant soft tissue spinal canal tumors. RESULTS: Diagnosis of soft tissue spinal canal malignancies starts with the identification of the lesion's spinal level and its relationship to the dura and medulla. The second step consists of characterizing it as likely-malignant based on radiological signs like a larger size, ill-defined margins, central necrosis, and/or increased vascularity. The third step is to identify additional imaging features such as intratumoral hemorrhage or cyst formation that can suggest specific malignancies. The physician can then formulate a differential diagnosis. The most encountered malignant soft tissue tumors of the spinal canal are anaplastic ependymomas, anaplastic astrocytomas, metastatic tumors, lymphoma, peripheral nerve sheath tumors, and central nervous system melanomas. A review of the imaging features of every type/subtype of lesion is presented in this work. Although magnetic resonance imaging remains the modality of choice for spinal tumor assessment, other techniques such as dynamic contrast agent-enhanced perfusion magnetic resonance imaging or diffusion-weighted imaging could guide diagnosis in specific situations. CONCLUSIONS: In this review, diagnostic strategies for several spinal cord tumors were presented, including anaplastic ependymoma, metastatic spinal cord tumors, anaplastic and malignant astrocytoma, lymphoma, malignant peripheral nerve sheath tumors , and primary central nervous system melanoma. Although the characterization of spinal cord tumors can be challenging, comprehensive knowledge of imaging features can help overcome these challenges and ensure optimal management of spinal canal lesions.

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.

Acute Macrocystic Thoracic Schwannoma: Systematic Review and Illustrative Case Example.

BACKGROUND: Schwannomas are benign peripheral nerve sheath tumors arising from myelinating Schwann cells. Although macrocystic changes are regularly encountered in schwannoma variants such as vestibular nerve tumors, they are exceedingly rare among spinal neoplasms. METHODS: Case report and systematic review of 4 databases (Ovid Medline, PubMed, Science Direct, and SCOPUS) from inception to present. All peer-reviewed publications reporting intradural cystic thoracic schwannoma were included. RESULTS: We identified 8 publications documenting 9 cases of cystic thoracic schwannoma. Four were female, 5 male; median age was 41 years (range, 27-80). Presentations ranged from incidental to pain, sensory changes, lower extremity paresis, or bowel/bladder dysfunction. Characteristic radiographic findings included T1 hypointensity, T2 hyperintensity, and cord effacement or compression. The present case followed a similar pattern: a 52-year-old male presented with worsening bilateral lower extremity weakness, low back pain, and gait dysfunction, worsening over 3 days. Examination also revealed decreased left lower extremity sensation. Imaging identified a well-delineated intradural, extramedullary macrocystic extending over T7-T10. The patient underwent a laminectomy resulting in complete tumor resection and restoration of intact neurologic function. Final pathology confirmed benign cystic schwannoma. CONCLUSIONS: Macrocystic thoracic schwannomas are exceedingly rare and lack a comprehensive scheme for clinical classification of their natural history and pathogenesis. We report the 10th case of such a schwannoma, and the first associated systematic review. Although macrocystic thoracic schwannomas are not frequently encountered, accurate diagnosis and appropriate neurosurgical treatment is critical in these vulnerable patients, given the opportunity for excellent functional outcomes following neurosurgical treatment.

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.

Exploring disparities in surgical recommendations for patients with primary intramedullary spinal cord tumors: an analysis of the Surveillance, Epidemiology, and End Results database from 2000 to 2019.

OBJECTIVE: Factors that may drive recommendations for operative intervention for patients with intramedullary spinal cord tumors (ISCTs) have yet to be extensively studied. The authors investigated racial and socioeconomic disparities in the management of patients with primary spinal cord ependymomas and nonependymal gliomas, with the aim of determining the associations between socioeconomic patient characteristics, survival, and recommendations for the resection of primary ISCTs. METHODS: The Surveillance, Epidemiology, and End Results registry was queried to identify all patients > 18 years of age with ISCTs diagnosed between 2000 and 2019. Univariable and multivariable logistic regression analyses were used to calculate odds ratios for variables associated with receiving a surgical recommendation. Log-rank tests and multivariable Cox proportional hazards models were used to investigate overall survival (OS) and disease-specific survival (DSS). RESULTS: The authors identified 2325 patients (mean age 49 [SD 16] years; 48.8% female; 67.4% non-Hispanic White, 7.8% non-Hispanic Black, 16.2% Hispanic, 6.5% Asian/Pacific Islander, 0.6% Native American; 56.7% married; 64.4% with household income < $75,000; 73.8% with spinal ependymoma; and 26.2% with nonependymal spinal glioma). Eighty-seven percent of patients received a surgical recommendation. In multivariable models, marriage was associated with higher odds of receiving a surgical recommendation for ependymomas (OR 1.80, p = 0.005). In multivariable models for nonependymal spinal gliomas, older age (OR 0.98, p = 0.001) and increased number of tumors (OR 0.62, p = 0.015) were associated with decreased odds of receiving surgical recommendations. Among ependymomas, marriage (HR 0.59, p = 0.001), younger age (HR 0.93, p < 0.001), female sex (HR 0.43, p = 0.006), and decreased number of tumors (HR 0.56, p < 0.001) were associated with improved OS. Among nonependymal spinal gliomas, median household income ≥ $75,000 (HR 0.69, p = 0.020) and younger age (HR 0.98, p < 0.001) were associated with improved DSS, while Black race (HR 4.65, p = 0.027) and older age (HR 1.05, p < 0.001) were associated with worse OS. CONCLUSIONS: In patients with spinal ependymomas and nonependymal spinal gliomas, recommendations for surgery appear to be unaffected by patient sex, race, or income. Survival disparities appear to exist among unmarried, male, Black, and lower-income cohorts. Continued initiatives to identify drivers of disparities while improving health equity in this patient population are needed.

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.