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Introduction: The incidence of hemangioblastoma is low, constituting only 1-5% of all spinal cord tumors. Specifically, intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome represents an exceedingly rare condition. Methods: We report the first documented case of cervical intradural extramedullary hemangioblastoma in China. A 53-year-old male patient presented with a 3-year history of mild right hemiplegia, segmental muscle strength and sensation impairment, and a positive pyramidal tract sign. MRI showed an abnormal oval signal focus in the intradural and extramedullary region at the C6-C7 vertebral level. Before surgery, angiography was performed to identify the supplying arteries and draining veins. Subsequent interventional therapy achieved over 90% occlusion of blood vessels, creating optimal conditions for complete resection of the spinal tumor. Results: The patient demonstrated satisfactory postoperative recovery with significant restoration of sensory and motor functions. Pathological examination showed a significant upregulation of CD31 in tumor cells and a substantial presence of the neuro-specific marker S100 in the tumor stroma, consistent with the diagnostic criteria for spinal hemangioblastoma. Conclusion: The rarity of cervical intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome was reaffirmed by a comprehensive review of the existing literature. Complete tumor resection remains the optimal approach for managing this uncommon condition, generally resulting in a favorable prognosis. Traditional open fenestration surgery is linked to elevated risks of bleeding and trauma. Meanwhile, endovascular injection of embolic agents may lead to residual lesions and an increased risk of recurrence. Therefore, we recommend a one-time combined treatment conducted in a hybrid operating room to achieve complete resection and effectively reduce intraoperative bleeding risk. Despite presenting challenges and requiring high proficiency, we still recommend this type of combined surgery as a suitable therapeutic option for such diseases. [ABSTRACT FROM AUTHOR]

IntroductionThe incidence of hemangioblastoma is low, constituting only 1-5% of all spinal cord tumors. Specifically, intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome represents an exceedingly rare condition.MethodsWe report the first documented case of cervical intradural extramedullary hemangioblastoma in China. A 53-year-old male patient presented with a 3-year history of mild right hemiplegia, segmental muscle strength and sensation impairment, and a positive pyramidal tract sign. MRI showed an abnormal oval signal focus in the intradural and extramedullary region at the C6-C7 vertebral level. Before surgery, angiography was performed to identify the supplying arteries and draining veins. Subsequent interventional therapy achieved over 90% occlusion of blood vessels, creating optimal conditions for complete resection of the spinal tumor.ResultsThe patient demonstrated satisfactory postoperative recovery with significant restoration of sensory and motor functions. Pathological examination showed a significant upregulation of CD31 in tumor cells and a substantial presence of the neuro-specific marker S100 in the tumor stroma, consistent with the diagnostic criteria for spinal hemangioblastoma.ConclusionThe rarity of cervical intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome was reaffirmed by a comprehensive review of the existing literature. Complete tumor resection remains the optimal approach for managing this uncommon condition, generally resulting in a favorable prognosis. Traditional open fenestration surgery is linked to elevated risks of bleeding and trauma. Meanwhile, endovascular injection of embolic agents may lead to residual lesions and an increased risk of recurrence. Therefore, we recommend a one-time combined treatment conducted in a hybrid operating room to achieve complete resection and effectively reduce intraoperative bleeding risk. Despite presenting challenges and requiring high proficiency, we still recommend this type of combined surgery as a suitable therapeutic option for such diseases.

Contemporary literature lacks examples of intradural, extramedullary spinal glomangiomas. Moreover, glomus tumors in general are exceedingly rare among benign spinal tumors and are mostly located within epidural space or within intervertebral foramen, and only a few cases have been documented to date. This report provides a detailed analysis of the clinical presentation, imaging characteristics, surgical intervention, and pathological findings of a 45-year-old patient experiencing progressive locomotor deterioration. The tumor was surgically excised, and subsequent histological examination identified it as a representative of glomus tumors—a glomangioma. Notably, this represents a unique case as it was the first example of such a tumor being discovered intradurally. Radical surgical excision remains the modality of choice in most benign spinal tumors of this localization. Although the malignant transformation of glomus tumors within the spine has not been documented thus far, cases have arisen in other areas. Consequently, we will investigate potential oncological treatments for cases with malignant potential and highlight advancements in surgical techniques for benign intradural spinal tumors. [ABSTRACT FROM AUTHOR]

Wei Peng, Yin Zhuang, Wei Cui, Wenjin Chen, Rupeng Chu, Zhenzhong Sun, Shujun Zhang Department of Spine Surgery, Wuxi Ninth People’s Hospital Affiliated to Soochow University, Wuxi, Jiangsu, People’s Republic of ChinaCorrespondence: Shujun Zhang, Department of Spine Surgery, Wuxi Ninth People’s Hospital Affiliated to Soochow University, No. 999 Liangqing Road, Binhu District, Wuxi, Jiangsu, People’s Republic of China, Tel +86-13861741839, Email spine_zhang@vip.163.comAbstract: This study describes a patient with an intradural extramedullary (IDEM) tumor removed entirely using the unilateral biportal endoscopic technique (UBE), achieving satisfactory clinical outcomes. A 60-year-old woman had a diagnosis of meningioma with sensations and motor dysfunction in the lower extremities and perineum and gait disturbances for three years, which has worsened over the last month. Preoperative imaging data showed a sizeable IDEM tumor at the T10 level, significantly compressing the thoracic spinal cord to the right side, with 80% intraspinal encroachment. The IDEM tumor was removed entirely by UBE surgery. To the best of our knowledge, this study may be the first to report the application of UBE techniques for IDEM tumor treatment. In this case, UBE provides a magnified and clear surgical field, greater maneuverability, and a less invasive surgical procedure. The procedure objectives were pathological confirmation, spinal cord decompression, and complete tumor removal; all were met. The patient was satisfied with her dramatically improved clinical symptoms. UBE may be an alternative surgical treatment option for benign IDEM tumors presenting with symptomatic, especially the non-giant lateral and posterior tumors.Keywords: unilateral biportal endoscopy, endoscopic spine surgery, intradural extramedullary, tumor, meningioma

Intradural extramedullary Ewing's sarcoma (IDEM) is a rare form of spinal tumor that requires a multidisciplinary approach and has high rates of recurrence and metastasis. This case report describes a 28-year-old male who presented with two months history of back pain and with sudden exacerbation and acute onset rapidly progressing lower limb weakness and numbness below the umbilicus with bowel and bladder incontinence. MRI spine showed a well-defined homogeneously enhancing IDEM at D11–D12 vertebral level with the lesion pushing the spinal cord to the right side. The patient underwent laminectomy and excision of the lesion, and histopathology confirmed Ewing's sarcoma. He received adjuvant chemotherapy and radiotherapy based on multidisciplinary team advice, and at 12 months follow-up, he was able to walk on his own and carry out his daily activities with minimal support. This case emphasizes the importance of early diagnosis and multidisciplinary management in improving the prognosis of intradural extramedullary Ewing's sarcoma. Given the rarity of this lesion, further research is needed to standardize its management. This report also includes a review of the existing literature on intradural extramedullary Ewing's sarcoma, highlighting the clinical features, radiological findings, treatment options, and outcomes.

Contemporary literature lacks examples of intradural, extramedullary spinal glomangiomas. Moreover, glomus tumors in general are exceedingly rare among benign spinal tumors and are mostly located within epidural space or within intervertebral foramen, and only a few cases have been documented to date. This report provides a detailed analysis of the clinical presentation, imaging characteristics, surgical intervention, and pathological findings of a 45-year-old patient experiencing progressive locomotor deterioration. The tumor was surgically excised, and subsequent histological examination identified it as a representative of glomus tumors—a glomangioma. Notably, this represents a unique case as it was the first example of such a tumor being discovered intradurally. Radical surgical excision remains the modality of choice in most benign spinal tumors of this localization. Although the malignant transformation of glomus tumors within the spine has not been documented thus far, cases have arisen in other areas. Consequently, we will investigate potential oncological treatments for cases with malignant potential and highlight advancements in surgical techniques for benign intradural spinal tumors.

Atypical teratoid rhabdoid tumors (ATRTs) are rare embryonal tumors comprising 1-2% of all pediatric CNS neoplasms. Spinal ATRTs are even more uncommon, accounting for 2% of all reported ATRT cases. Despite their rarity, ATRTs affect young children disproportionately and are characterized by a high malignant potential due to a heterogeneous cellular composition and inactivating mutations in the SMARCB1 (90%) and SMARCA4 (10%) genes. A 15-month-old female presented with a 2-week history of decreased lower extremity movement and new-onset need for assistance with ambulation. MRI lumbar spine revealed a contrast-enhancing intradural mass at the L3-L4 level with iso-intensity on T1 and T2 sequences. The patient subsequently underwent subtotal tumor resection (∼80%) given concerns for maintaining neurological function. Final pathology was consistent with spinal ATRT, and she later underwent adjuvant chemoradiation therapy per ACNS0333 protocol. She has since remained in remission with age-appropriate developmental milestones over the past 2 years. ATRTs should be considered in the differential diagnosis of intradural spinal lesions, especially in the pediatric patient population. Clinical course, presentation, and diagnosis is often delayed due to the rarity of these tumors, but contrasted craniospinal MRI is key for diagnosis and histopathology with IHC staining showing loss of INI is confirmatory. While gross total resection is the goal, maximal safe tumor resection should be prioritized in order to preserve neurological function. Adjuvant chemoradiation following gross total/subtotal resection has been shown to significantly improve overall survival. [ABSTRACT FROM AUTHOR]

Study design: Retrospective cohort study using prospectively collected registry data. Objective: The purpose of this study is to evaluate health-related quality of life (HRQOL) and postoperative satisfaction in patients with different histotypes of benign extramedullary spinal tumors (ESTs). Background: Little is known about how different histotypes influence HRQOL and postoperative satisfaction in EST patients. Materials and Methods: Patients undergoing primary benign EST surgery at 11 tertiary referral hospitals between 2017 and 2021 who completed preoperative and 1-year postoperative questionnaires were included. HRQOL assessment included the Physical Component Summary and Mental Component Summary (MCS) of Short Form-12, EuroQol 5-dimension, Oswestry/Neck Disability Index (ODI/NDI), and Numeric Rating Scales (NRS) for upper/lower extremities (UEP/LEP) and back pain (BP). Patients who answered "very satisfied," "satisfied," or "somewhat satisfied" on a seven-point Likert scale were considered to be satisfied with treatment. Student ttests or Welch's t-test were used to compare continuous variables between two groups, and one-way analysis of variance was used to compare outcomes between the three groups of EST histotypes (schwannoma, meningioma, atypical). Categorical variables were compared using the χ² test or Fisher exact test. Results: A total of 140 consecutive EST patients were evaluated; 100 (72%) had schwannomas, 30 (21%) had meningiomas, and 10 (7%) had other ESTs. Baseline Physical Component Summary was significantly worse in patients with meningiomas (P=0.04), and baseline NRS-LEP was significantly worse in patients with schwannomas (P=0.03). However, there were no significant differences in overall postoperative HRQOL or patient satisfaction between histology types. Overall, 121 (86%) patients were satisfied with surgery. In a subgroup analysis comparing intradural schwannomas and meningiomas adjusted for patient demographics and tumor location with inverse probability weighting, schwannoma patients had worse baselineMCS (P=0.03), ODI (P=0.03), NRS-BP (P<.001), and NRSLEP (P=0.001). Schwannoma patients also had worse postoperative MCS (P=0.03) and NRS-BP (P=0.001), with no significant difference in the percentage of satisfied patients (P=0.30). Conclusions: Patients who underwent primary benign EST resection had a significant improvement in HRQOL postoperatively, and ~90% of these patients reported being satisfied with their treatment outcomes one year after surgery. EST patients may exhibit a relatively lower threshold for postoperative satisfaction compared with patients undergoing surgery for degenerative spine conditions. [ABSTRACT FROM AUTHOR]

Primary intradural extramedullary Ewing sarcoma (IEES) is the rarest type of Ewing sarcoma. Extreme caution is required for the diagnosis of IEES because benign intradural spinal tumors can be mistaken for IEES in the early stages of imaging and clinical evaluation. IEES tumors have no standardized treatment guidelines because of the lack of research on the therapeutic aspects of these tumors. Herein, we present a case of primary IEES in a male adolescent with a fast progression of the disease. Diagnosis of IEES was suspected with imaging (computed tomography scan and lumbosacral magnetic resonance imaging) and was confirmed with histology and immunohistochemistry (positive reactivity for CD99 and FLI1). He was successfully treated with surgical intervention with no radiotherapy or chemotherapy. Imaging studies are helpful in making the initial diagnosis of intradural extramedullary Ewing sarcoma. Surgery is considered to be a successful method of treatment for this condition.

Spinal intradural extramedullary teratomas are rare tumors arising from more than one germ cell layers and are more common in children than in older age groups. Teratomas are more commonly seen in intracranial compartment and are extremely rare in the spine. They are difficult to diagnose pre-operatively and the definitive diagnosis is usually done by histopathologic evaluation. Intraspinal teratomas should be considered in the differential diagnosis of any intradural extramedullary tumor of the spine. We reviewed the literature and reported a rare case of intradural extramedullary teratoma in an adolescent located in dorsolumbar spine without any history of prior spinal procedure. [ABSTRACT FROM AUTHOR]

Background: Glomus tumors are very infrequent in the spine where they can grow intraosseously at any level. We were able to identify only eight such cases in the literature, with only one occurring in the sacrum. Here, a 48-yearold male with sacral S1/S2 radiculopathy was found to have a rare glomangioma/glomus tumor of the sacral region. Case Description: A 48-year-old male presented with left-sided S2 radiculopathy characterized by left lower extremity weakness/paresis. The magnetic resonance showed an intradural extramedullary mass measuring 1.8 × 1.9 × 4.3 cm at S1-S2 extending through the left foramen, inhomogeneously enhanced with contrast. He underwent an S1-S2 durotomy with gross total excision of the mass. Pathologically, it proved to be a glomus tumor. Two months postoperatively, he ambulated without the support and demonstrated no tumor recurrence at 1 postoperative year. Conclusion: Glomus tumors involving the sacral region are rare and can be successfully excised resulting in good clinical outcomes. [ABSTRACT FROM AUTHOR]

The development of minimally invasive surgery has promoted the use of the paraspinal mini-tubular technique (PMTT) for spinal tumors. The similarity of the efficacy of PMTT to that of traditional surgery remains unclear; randomized controlled trials (RCTs) have not been conducted to investigate this technique for spinal tumors. The conventional surgery used for such tumors is laminoplasty (LP). To compare the differences between the two surgical techniques, an RCT is significantly required. Therefore, a prospective, multi-center, non-inferiority RCT was designed to compare the safety and effectiveness of LP and PMTT for treating spinal intradural extramedullary (IDEM) tumors. Moreover, the availability of PMTT, including its advantages and disadvantages, surgical indications, procedures, complications, and prognosis, would be explored. Overall, 280 patients will be randomly allocated to the PMTT and LP groups in a 1:1 ratio. The trial hypothesis is that PMTT has superior or equivalent efficacy and cost-effectiveness to LP. The primary outcome is the Japanese Orthopedic Association score. The non-inferiority margin for the primary outcome is five. The Ethics Committee of Fujian Medical University Union Hospital, Fuzhou, China, has approved this study (project number: FJMUUH05). Any results of the trial will be published in international peer-reviewed journals and disseminated through presentations at scientific conferences.Trial registration numberChiCTR2100047582

Anterior intradural extramedullary meningiomas are intimidating, especially in the thoracic spine due to the spinal cord impeding conventional surgical corridor, restricted space, and precarious cord vascularity. 1,2 This is possibly the first report in the literature of a minimally invasive surgery: far-lateral hybrid exoscopic and angled endoscopic approach for an anterior thoracic spine meningioma. A 60-year-old woman presented with an anterior T1-2 meningioma with a flattened cord draping over the tumor more toward the right, leaving a potential corridor on the left lateral aspect (Video 1). Under a three-dimensional exoscope, a minimally invasive retractor was placed through the left paramedian incision centered on the lamina–transverse process junction; lateral fenestration and T1-2 facetectomy were carried out with a high-speed drill and rongeurs, meticulously preserving C7-T1 articulations. 3,4 Following coagulation of the anterolateral dura over the tumor, a lateral longitudinal durotomy with a T-shaped anterolateral extension was performed around the tumor. Following devascularization, disconnection, and extra-arachnoidal dissection, a unique lateral rolling technique was employed, wherein the tumor was bimanually rolled onto the bony gutter created earlier without disturbing the spinal cord. This was followed by coagulation and excision of the dural tail. A 45° endoscope was then inserted to look for any residual tumor or dural tail. 5 As the durotomy was lateral with excision of the dural tail, a multilayered soft tissue closure without dead space was sufficient to prevent cerebrospinal fluid leak. 3 Fixation was not needed as only a single thoracic spine facet was disrupted. 4 The patient recovered fully with radiology confirming total excision. This minimally invasive lateral rolling technique is safe and effective for anterior meningioma with the ergonomics of exoscope & the angled optics of endoscope complementing each other. [ABSTRACT FROM AUTHOR]

Simple Summary: Renal cell carcinoma is a highly metastatic tumor, mainly to the lungs (50%), bone (49%), lymph-nodes (6–32%), liver (8%), and brain (3%). A wide and accurate literature review has disclosed only 51 cases of intradural spinal metastasis from sporadic renal cell carcinoma, of which 32 at intramedullary and 19 at extramedullary localizations. Once detected, they represent a sign of advanced disease and often lead to rapidly progressive neurological deficits. Because of these few reported data, there are no defined guidelines of treatment and the decision making in the choice of the best strategy should consider the curative, functional and palliative aspects, accordingly the management should be tailored for each patient. The options include surgery, radiotherapy, and chemotherapy, which can be performed in isolation or various combinations at the discretion of each institution. We discuss the role of surgery in the management of spinal intradural metastases from renal cell carcinoma. Background: Due to the few reported cases of spinal intradural metastases from renal cell carcinoma (RCC), there is no unanimous consensus on the best treatment strategy, including the role of surgery. Methods: A wide and accurate literature review up to January 2022 has disclosed only 51 cases of spinal intradural metastases from RCC. Patients with extramedullary (19) and those with intramedullary (32) localization have been separately considered and compared. Demographics, clinical, pathological, management, and outcome features have been analyzed. Results: Extramedullary lesions more frequently showed the involvement of the lumbar spine, low back pain, and solitary metastasis at diagnosis. Conversely, the intramedullary lesions were most often detected in association with multiple localizations of disease, mainly in the brain. Surgery resulted in improvement of clinical symptoms in both groups. Conclusion: Several factors affect the prognosis of metastatic RCC. The surgical removal of spinal metastases resulted in pain relief and the arresting of neurological deficit progression, improving the quality of life and overall survival of the patient. Considering the relative radioresistant nature of the RCC, the surgical treatment of the metastasis is a valid option even if it is subtotal, with a consequent increased risk of recurrence, and/or a nerve root should be sacrificed. [ABSTRACT FROM AUTHOR]

Synovial sarcoma (SS) accounts for 5- 10% of all adult soft-tissue sarcomas and only 5% arises in the spine. It presents like any other spinal tumor, namely axial pain with symptoms due to neural compression. Imaging findings can also be similar to any other benign spinal tumor. We present a 43-year-old male who presented with symptoms of radiculopathy and neural compression and imaging revealed a dumbbell tumor at C6 to D1 with transforaminal and paraspinal extension on the right side. After surgical excision the histopathology revealed a rare synovial sarcoma of the spine. Synovial sarcoma of the spine though rare, are difficult to differentiate based on their presentation and imaging characteristics from benign spinal tumors. However, subtle findings on imaging and a pre-operative biopsy may aid in performing a more definitive surgery upfront rather than a re-do surgery after the histopathological diagnosis.

Study Design A retrospective study was done to assess the outcome of the new technique of flipped reposition laminoplasty for excision of intradural extramedullary (IDEM) spinal tumors of the thoracolumbar region. Purpose To describe flipped reposition laminoplasty technique and evaluate its outcomes. Overview of Literature Laminectomy has been the conventional approach for the surgical excision of IDEM spinal tumors, but it has potential postoperative complications. Laminoplasty maintains the posterior arch of the spine and avoids complications seen in Laminectomy, such as instability, epidural scarring, and kyphotic deformity. Methods Fourteen patients (nine females and five males) diagnosed with IDEM tumors of the thoracolumbar region operated between 2016 and 2018 were included in this study. Pathologically, five cases were schwannomas; four cases were meningiomas; two cases were ependymomas; and one case each was lymphoma, neurofibroma, and teratoma. All patients had their neurological deficits documented using the American Spinal Injury Association (ASIA) impairment scale. After completion of all preanesthetic formalities, the patients were operated upon by a single surgeon using the flipped reposition laminoplasty technique. Follow-up was done at 1, 3, 6, and 12 months post operation and yearly thereafter. Results The mean age of the patients was 35.28 years (14–65 years), and the mean follow-up duration was 17 months (6–26 months). Two patients were assessed with ASIA grade A neurology, one patient improved to ASIA grade B, whereas the other did not improve. Two patients improved from ASIA grade B to ASIA grade D, and seven patients with ASIA grades C and D improved to ASIA grade E. Fusion at the osteotomy site was seen in 92.85% (13 out of 14) cases on one side within 6 months post operation. Fusion was seen in all the cases within 1 year post operation. Conclusions Flipped reposition laminoplasty is an excellent technique providing adequate exposure and additional stability postoperatively.

Background: Cavernous malformations (CMs) are vascular malformations that usually occur in the brain and occasionally in the spine. Most CMs are sporadic, but they can also be caused by heterozygous cerebral cavernous malformation (CCM) gene mutations. This case describes an intradural extramedullary CM in a unique location, within a fascicle of the S1 nerve root. This tumor was found to have a CCM2 mutation. An operative video demonstrates the surgical procedure used to remove this S1 intradural extramedullary CM., Observations: A 61-year-old male with an intradural extramedullary CM, located within a fascicle of the S1 nerve root, presented with pain and neurological symptoms. Additional imaging revealed two CCMs. The S1 CM was removed in toto. Subsequently, genetic screening revealed a CCM2 mutation., Lessons: Given the rarity of a radicular CM, diagnosis can be challenging. CMs should be considered in patients with radicular pain and corresponding nerve root thickening. When a CM is suspected, imaging of the complete neuraxis should be performed. If multiple CMs are found, genetic screening is indicated. Complete resection of the CM yields good clinical results. The operative video in this case might help surgeons encountering this rare pathology to prepare for surgery. https://thejns.org/doi/10.3171/CASE24270.

Background: Granular cell tumors (GCTs) are uncommon peripheral nerve sheath tumors of Schwann cell origin that may occur throughout the body. However, they rarely occur in the spinal canal. Case Description: A 49-year-old male presented with burning sensation in the left knee. The MRI of the lumbar spine showed an L3-L4 intradural extramedullary tumor. Complete surgical resection was successfully performed and the L3 root burning improved. Histopathologically, the lesion proved to be a benign GCT. Conclusion: Spinal GCTs are rare benign tumors that may be found in an intradural extramedullary location in the spine. The preferred treatment is complete surgical resection as subtotal/partial resection may result in recurrence warranting radiation therapy. [ABSTRACT FROM AUTHOR]

Intradural extramedullary bronchogenic cysts (IEBC) are rare congenital cystic lesions. The clinical manifestations, radiological characteristics, especially the optimal treatment regimen are not well-understood. We retrospectively analyzed a series of patients with confirmed IEBC in Tangdu hospital and reviewed the published works to gain a comprehensive understanding of IEBC. In our institution, nine consecutive patients had pathologically confirmed IEBC between 2005 and 2018. We also identified 27 patients from previous studies. The most common presentations on magnetic resonance imaging (MRI) were hypointensity on T1-weighted images (T1WI), hyperintensity on T2-weighted images(T2WI), and no improvement on T1WI contrast-enhanced with gadolinium (94.4%). All patients in our center and the patients we reviewed received surgical resection; gross total resection (GTR) and partial resection (PR) were achieved in 20 (55.6%) and 16 (44.4%) patients, respectively. The symptom remission rate of patients who underwent GTR was 100%, which was similar to those who underwent PR (93.8%) (P = 0.457). The recurrence rate was 12.5% in the group who underwent PR and nil after GTR (P = 0.202). According to our current investigation, the surgical resection degree is irrelevant to the symptom remission rate. Therefore, we suggest that total resection should not be recommended for cases with tight adhesion. For patients with PR, longer follow-up will be necessary to determine the long-term outcome. [ABSTRACT FROM AUTHOR]

Intradural extramedullary bronchogenic cysts (IEBC) are rare congenital cystic lesions. The clinical manifestations, radiological characteristics, especially the optimal treatment regimen are not well-understood. We retrospectively analyzed a series of patients with confirmed IEBC in Tangdu hospital and reviewed the published works to gain a comprehensive understanding of IEBC. In our institution, nine consecutive patients had pathologically confirmed IEBC between 2005 and 2018. We also identified 27 patients from previous studies. The most common presentations on magnetic resonance imaging (MRI) were hypointensity on T1-weighted images (T1WI), hyperintensity on T2-weighted images(T2WI), and no improvement on T1WI contrast-enhanced with gadolinium (94.4%). All patients in our center and the patients we reviewed received surgical resection; gross total resection (GTR) and partial resection (PR) were achieved in 20 (55.6%) and 16 (44.4%) patients, respectively. The symptom remission rate of patients who underwent GTR was 100%, which was similar to those who underwent PR (93.8%) (P = 0.457). The recurrence rate was 12.5% in the group who underwent PR and nil after GTR (P = 0.202). According to our current investigation, the surgical resection degree is irrelevant to the symptom remission rate. Therefore, we suggest that total resection should not be recommended for cases with tight adhesion. For patients with PR, longer follow-up will be necessary to determine the long-term outcome.

Background: IgG4-related disease (IgG4RD) is a multisystemic progressive fibroinflammatory and lymphoproliferative autoimmune disorder of unknown etiopathogenesis; and its occurrence in intradural extramedullary (IDEM) location is extremely rare.Aim and Objective: The aim of this study was to review all IDEM IgG4RD cases described in English literature and to signify the role of surgery in its management at this rare location along with the surgical technique and intraoperative findings.Methods: An Internet-based search (PubMed) for the published reports describing spinal cases of IgG4RD with IDEM involvement was done using the keyword: spinal IgG4-related disease. An illustration of a case of cranial IgG4-related disease in a young man who developed metachronous cervical involvement in intradural extramedullary (IDEM) location is also presented.Results: Amongst 45 spinal IgG4RD cases reported in English Literature, only 3 cases were IDEM IgG4RD. In the illustrated case, portion of the cervical lesion causing cervical cord encasement was intradural but extraarachnoidal- located between arachnoid and dura, without any pial involvement. All these 4 cases improved following surgery.Conclusion: Timely meticulous resection of mass lesion in situations of medical treatment failure or progressive neurological decline can lead to reversibility of mass effect-associated neurological manifestations of IgG4RD. [ABSTRACT FROM AUTHOR]

Abstract Background As chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy. A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up. The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates. Case presentation A 43-year-old woman presented with an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy, C3-5 lateral mass screw instrumentation, and mass resection. All symptoms resolved by the 12-month postoperative follow-up visit. Conclusions This is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature. Unfortunately, the very rare form of extraosseous intradural chordoma is poorly understood: the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.

Background: Spinal tumor is a common cause of morbidity in otherwise healthy population, timely diagnosis and treatment of spinal tumor gives excellent outcome. Aims and Objective: We report experience and clinical outcomes of 75 cases with Intradural extramedullary meningiomas operated in last 21 years. Materials and Methods: All the patients were clinically assessed with Nurick’s Grading (both pre and post operatively). MRI was the main armamentarium for operating planning. In all the patients dural attachments were coagulated without any dural excision. Results: Out of 75 patients, 65% were female. Peak incidence was noted in 4th & 5th decade and majority of patients having tumor in the thoracic spine and lateral to the cord. The entire patient showed remarkable clinical improvement according to Nurick’s grade. Total removal was achieved in 69 (90.2%) patients. Two patients had re-growth of tumor in 1 yr. follow up. No postoperative mortality noted in the present series. Conclusion: Spinal meningioma excision without dural resection did not show any increase in recurrences.

Background: Cervical spine schwannomas are uncommon. Sudden onset quadriparesis has been reported in cases of acute hemorrhage within cervical spine schwannoma; however, no such case was found in the literature describing quadriplegia as a result of neck manipulation. We present a case of a sudden onset, temporary loss of SSEP and MEP due to positional neck changes.Case presentation: A 29-year-old male patient presented with a three days onset right-sided weakness progressing to the left side with an associated fecal and urinary incontinence a few days. MRI with and without contrast for the brain and the whole spine was done and it revealed an IDEM lesion at C2-C4 that is iso to hyper-intense on T2 with avid contrast enhancement on T1. While preparing the patient for surgery and during positioning, SSEP and MEP signals were lost and subsequently restored when the neck was positioned with a mild extension. Laminectomy of the C2-C3-C4 levels was done, and the tumor was completely resected. Frozen section revealed a diagnosis of schwannoma. Post-operatively, the patient improved significantly and gained his motor and sensory functions. Conclusion: Sudden Loss of MEP and SSEP signals with neck positioning in an IDEM lesion is uncommon. We hypothesize that positional changes in the cervical spine lead to continuous and aggravated pressure on the spinal cord, albeit temporary. Manipulating the neck to its original position can decompress the spinal cord and restore the original neurophysiological status.

Background: Despite technological advances in medical treatment, the prognosis of the rarely reported spinal subdural abscesses (SSAs) has remained a serious entity largely unaffected, especially when they are diagnosed late. In this study, the authors aimed to present the surgical outcomes of 3 consecutive pediatric patients with SSA. Materials and Methods: We retrospectively reviewed the medical records of pediatric patients with spinal lesions who underwent surgery at 2 neurosurgical centers spanning 7 years, from 2012 to 2019. All pediatric patients who were diagnosed with SSA (n = 3) were selected as the core sample for this study. Results: Three pediatric patients (2 females and 1 male) with SSA were surgically treated. Holocord SSA was observed in 1 patient. The mean age was 7.1 ± 7.7 years. The most common presenting symptoms were gait disturbance and weakness of lower extremities (100%). The mean preoperative course was 5.7 ± 4.0 weeks. The causative pathogens were Escherichia coli (E. coli) and M. tuberculosis. In the 2nd case, the pathogen was non-tuberculosis mycobacterium in the extramedullary abscess. In the 44th postoperative month, she underwent surgery for intramedullary abscess. The causative pathogen was E. coli. Except for 1 male adolescent who presented with severe clinical status (paraplegic), the improvement was observed in all patients at their last follow-up after 50.3 ± 43.5 months of average. Conclusions: Drainage followed by appropriate antibiotics is the optimal treatment for SSAs. Early diagnosis and urgent surgical treatment are essential for a good prognosis. All surgically treated SSA patients with neurological deficits were rehabilitated with physical therapy postoperatively. [ABSTRACT FROM AUTHOR]

Synovial sarcoma (SS) accounts for 5- 10% of all adult soft-tissue sarcomas and only 5% arises in the spine. It presents like any other spinal tumor, namely axial pain with symptoms due to neural compression. Imaging findings can also be similar to any other benign spinal tumor. We present a 43-year-old male who presented with symptoms of radiculopathy and neural compression and imaging revealed a dumbbell tumor at C6 to D1 with transforaminal and paraspinal extension on the right side. After surgical excision the histopathology revealed a rare synovial sarcoma of the spine. Synovial sarcoma of the spine though rare, are difficult to differentiate based on their presentation and imaging characteristics from benign spinal tumors. However, subtle findings on imaging and a pre-operative biopsy may aid in performing a more definitive surgery upfront rather than a re-do surgery after the histopathological diagnosis. [ABSTRACT FROM AUTHOR]

Melanocytic lesions of the nervous system are thought to arise from leptomeningeal melanocytes, which are derived from neural crest and include diffuse melanocytosis, melanocytomas, and malignant melanomas. Meningeal melanocytomas are extremely rare benign lesions. The usual treatment of intradural extramedullary melanocytomas involves surgical removal through a posterior approach using a laminectomy or laminotomy. We present a 30-year-old female harboring a C6-T1 ventrally located intradural extramedullary lesion compressing the cord anteriorly. The lesion was totally resected via an anterior approach with oblique corpectomy even if the usual treatment involves surgical removal through a posterior approach using a laminectomy or laminotomy. There is no evidence of recurrence at 4-year follow-up records of the patient. We discuss the surgical approach of these rare lesions. [ABSTRACT FROM AUTHOR]

Intradural extramedullary (IDEM) metastatic spine tumors of nonneurogenic origin, excluding leptomeningeal seeding, are very rare. Most patients with IDEM metastatic spine tumors of nonneurogenic origin have coexisting brain metastasis. We report a case of IDEM metastatic malignant melanoma without antecedent or coexisting brain metastasis. A 52-year-old man presented with back pain, numbness in the lower extremities, paraparesis, and residual urine sensation after voiding. The patient had a history of conjunctival melanoma in the left eye and underwent surgical removal 3 years 6 months ago. Whole-spine magnetic resonance imaging showed an IDEM tumor at the T11 and T12 level, with compression of the spinal cord. Total laminectomy and resection of the tumor was performed. Pathology confirmed a malignant melanoma. Postoperatively, adjuvant chemotherapy was performed. Pain and neurologic deficit gradually improved, but complete recovery was not achieved. Although rare, even without brain metastasis, malignant melanoma can cause IDEM metastatic malignant melanoma. For neurologic recovery and maintaining quality of life of the patient, surgical treatment for decompression of the spinal cord and postoperative adjuvant treatment for IDEM metastatic malignant melanoma should be considered unless multiple lesions are present in different parts within the spinal column. [ABSTRACT FROM AUTHOR]

Study Design: A retrospective study was done to assess the outcome of the new technique of flipped reposition laminoplasty for excision of intradural extramedullary (IDEM) spinal tumors of the thoracolumbar region. Purpose: To describe flipped reposition laminoplasty technique and evaluate its outcomes. Overview of Literature: Laminectomy has been the conventional approach for the surgical excision of IDEM spinal tumors, but it has potential postoperative complications. Laminoplasty maintains the posterior arch of the spine and avoids complications seen in Laminectomy, such as instability, epidural scarring, and kyphotic deformity. Methods: Fourteen patients (nine females and five males) diagnosed with IDEM tumors of the thoracolumbar region operated between 2016 and 2018 were included in this study. Pathologically, five cases were schwannomas; four cases were meningiomas; two cases were ependymomas; and one case each was lymphoma, neurofibroma, and teratoma. All patients had their neurological deficits documented using the American Spinal Injury Association (ASIA) impairment scale. After completion of all preanesthetic formalities, the patients were operated upon by a single surgeon using the flipped reposition laminoplasty technique. Follow-up was done at 1, 3, 6, and 12 months post operation and yearly thereafter. Results: The mean age of the patients was 35.28 years (14-65 years), and the mean follow-up duration was 17 months (6-26 months). Two patients were assessed with ASIA grade A neurology, one patient improved to ASIA grade B, whereas the other did not improve. Two patients improved from ASIA grade B to ASIA grade D, and seven patients with ASIA grades C and D improved to ASIA grade E. Fusion at the osteotomy site was seen in 92.85% (13 out of 14) cases on one side within 6 months post operation. Fusion was seen in all the cases within 1 year post operation. Conclusions: Flipped reposition laminoplasty is an excellent technique providing adequate exposure and additional stability postoperatively. [ABSTRACT FROM AUTHOR]

Dermoid cysts are benign congenital tumors that develop early in life. These tumors are classified by the presence of all 3 germ layers. Spinal intradural extramedullary teratoma is a rare disease, which is more common in children under 5 years of age than in adults. A 12-year-old girl with a dermoid cyst at the lower lumbar level presented with 2-month low back pain and intermittent lower extremity radicular symptoms on the right side. Magnetic resonance imaging scan of the spine revealed an intradural extramedullary mass lesion at L4-5. Surgical excision of the cyst was successfully performed. Surgical and histopathologic findings confirmed extramedullary ruptured matured teratoma. Postoperatively, the patient had remarkable clinical improvement. Although dermoid cysts are uncommon, they should be considered in the differential diagnosis of spinal lesions in patients with lower back pain. It can be successfully treated with surgical excision. [ABSTRACT FROM AUTHOR]

Primary spinal melanoma is too rare to have any systemic statistical data on the epidemiology of this disease. We report a 58-year-old woman presented with spastic gait for 10 days, preceded by chronic pain at her left anterior chest that progressively spread to her neck, left shoulder, and arm in half a year. Cervical magnetic resonance imaging (MRI) displayed a well-enhancing focal intraspinal extramedullary tumor at C6/7 level. After surgical resection of the tumor and thorough studies to exclude extraspinal melanoma, the primary spinal melanoma is diagnosed. Postoperative MRI did not disclose any evidence of recurrence at 4 and 21 months later. We present the clinical, radiological, and pathological features of a patient with a primary cervical extramedullary spinal melanoma.

Ossified spinal meningiomas are a rare form of spinal tumors. These tumors increase surgical morbidities due to their hard consistency and strong adhesion to the neural tissue and relatively narrow surgical space. Here, the authors describe the clinical findings, surgical strategies, and histological findings of a patient with an ossified meningioma. Preoperative diagnosis of these tumors can prevent surgical morbidities. Total resection can be curative with the application of meticulous microsurgical techniques.

Minimally invasive surgery (MIS) is increasingly being adopted for spinal intradural tumors. Through the use of conventional microscopy or exoscopy for large lobulated nerve sheath tumors, the posterior root attachment is often visualized only after mobilizing the tumor. Here, the authors describe the utility of angled endoscopy with its panoramic view for a T10 nerve sheath tumor. Gross-total extracapsular excision was achieved utilizing a minimally invasive right paraspinous approach, fenestration, lateral durotomy, sliding delivery of the tumor, sharp dissection of radicular attachments under neuromonitoring, and dural closure with oblique clips. Angled endoscopes help visualize the attachments behind large multilobulated tumors and confirm the totality of excision. The video can be found here: https://stream.cadmore.media/r10.3171/2024.1.FOCVID23214., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication.The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2024, The Authors.)

Background: Spinal tumor is a common cause of morbidity in otherwise healthy population, timely diagnosis and treatment of spinal tumor gives excellent outcome. Aims and Objective: We report experience and clinical outcomes of 75 cases with Intradural extramedullary meningiomas operated in last 21 years. Materials and Methods: All the patients were clinically assessed with Nurick's Grading (both pre and post operatively). MRI was the main armamentarium for operating planning. In all the patients dural attachments were coagulated without any dural excision. Results: Out of 75 patients, 65% were female. Peak incidence was noted in 4th& 5th decade and majority of patients having tumor in the thoracic spine and lateral to the cord. The entire patient showed remarkable clinical improvement according to Nurick's grade. Total removal was achieved in 69 (90.2%) patients. Two patients had re-growth of tumor in 1 yr. follow up. No postoperative mortality noted in the present series. Conclusion: Spinal meningioma excision without dural resection did not show any increase in recurrences. [ABSTRACT FROM AUTHOR]

Abstract Intradural Extramedullary Spinal Melanocytoma (IESM) is an extremely uncommon tumour arising from the spinal leptomeninges; both from a gross pathology and microscopic point of view it can mimick its malignant counterpart, the Melanoma. Such tumours are usually solitary, with a lower proliferating rate and without obvious SC invasion. Their common differential diagnoses include Spinal Schwannoma and Spinal Meningiomas since they share a significant amount of radiological similarities. It is a relatively benign condition which is, to date, with no more than 24 previously reported cases, yet widely unexplored and poorly understood. We report the detailed clinical history of a 60 years old individual suffering from IESM and, by means of a thorough Literature review, the most relevant features concerning the epidemiological issues, the clinical course, the radiological appearance, the surgical results and the typical gross and microscopic pathology of a cohort of previously reported cases of IESM are extensively discussed and systematically investigated through statistical analyses in order to add to the relevant Literature a dedicated work concerning this rare and enigmatic condition. [ABSTRACT FROM AUTHOR]

Background: Intradural extramedullary tuberculoma of the spinal cord (IETSC) is an extremely rare form of spinal tuberculosis (TB) that is believed to be due to a host's immune reaction against the Mycobacterium protein derivatives. Case Description: A 25-year-old male with human immunodeficiency virus, hepatitis C virus, and disseminated TB on antitubercular therapy for the past 8 months, presented with paraplegia of 2 months duration. When the MRI spine revealed multiple peripheral rim enhancing intradural extramedullary lesions from T6 to T8 and dorsally from T10 to T11, the patient was diagnosed with IETSC. At surgery, we countered cystic lesions adherent to the dura and the spinal cord, containing a whitish material. Postoperatively, the patient showed clinical improvement in motor power and sensation. Conclusion: Intradural extramedullary spinal tuberculomas in patients with a history of TB and spinal cord compression, although rare, should be considered among the differential diagnoses. [ABSTRACT FROM AUTHOR]

Introduction: Surgical outcome of spinal tumours varies depending on a number of factors such as: site of tumour compression within the spinal canal, the histological characteristics of tumours, the neurological progression and initial response to corticosteroid therapy, patient's age, comorbidity, tumour extension, involvement of neighbor structures and organs etc. Materials & Methods: The 46 patients with intradural extramedullary (IDEM) spinal tumour underwent surgery by our team in 7 years (2010-2017) were reviewed retrospectively. Discussion: Analysis of the surgical outcome of our spinal tumour patients was done on different variables like age, sex, presenting symptoms, neuro imaging, comorbidities etc. The aim of surgery was decompression of the spinal cord and total removal of the tumour. Conclusion: The aim of this study is to analyze the data to make conclusion for more effective strategy as per site, size, type, resectibility and histological variety to establish an effective treatment protocol and prevention of per-operative and post-operative complications. Intradural extramedullary tumor can be radically resected with no mortality and minimal peri-operative morbidity. [ABSTRACT FROM AUTHOR]

Primary spinal melanoma is too rare to have any systemic statistical data on the epidemiology of this disease. We report a 58-year-old woman presented with spastic gait for 10 days, preceded by chronic pain at her left anterior chest that progressively spread to her neck, left shoulder, and arm in half a year. Cervical magnetic resonance imaging (MRI) displayed a well-enhancing focal intraspinal extramedullary tumor at C6/7 level. After surgical resection of the tumor and thorough studies to exclude extraspinal melanoma, the primary spinal melanoma is diagnosed. Postoperative MRI did not disclose any evidence of recurrence at 4 and 21 months later. We present the clinical, radiological, and pathological features of a patient with a primary cervical extramedullary spinal melanoma. [ABSTRACT FROM AUTHOR]