Simultaneous spine extradural and intradural teratomas in a pediatric patient: A rare presentation with insights in the flawed migration of germ cells theory.

Spinal teratomas are infrequent lesions in the pediatric population. These lesions can be extradural, intradural or intramedullary. We present a case of an 8-month-old boy that was assessed for underdevelopment of motor milestones. The neurologic examination revealed hyporeflexia, decreased sensation and flaccid paraplegia. MRI of the spine revealed two simultaneous and independent lesions in the extradural and intradural compartment. A laminectomy was performed for the T4-T7 vertebrae with total resection of both lesions. The histopathological analysis confirmed both lesions to be mature cystic teratomas. At the 1-year follow-up, the patient remained with no recovery of neurological function. A debate takes place regarding the etiology of formation of these lesions in the spine. The simultaneous presentation of two independent lesions in this patient could contribute to define the flawed migration of germ cells theory as the etiology for formation of teratomatous lesions in the spine.

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.

Anterior clinoidectomy in low lying internal carotid artery-posterior communicating artery aneurysm.

BACKGROUND: At times, a regulation internal carotid artery-posterior communicating artery junction (ICA-P-Comm) aneurysm becomes a surgical hurdle owing to its close proximity to the anterior clinoid process, an immovable ICA and a concealed dominant P-Comm artery arising from the aneurysm neck. METHOD: A 70 year old patient with a low lying ICA-P-Comm aneurysm underwent a "tailored" intradural clinoidectomy for aneurysm clipping. CONCLUSION: A tailored anterior clinoidectomy to expose "just enough" allows a proximal ICA control in a suitable area, mobility of an atherosclerotic ICA and exposes the P-Comm artery origin which are essential in safe clipping of these aneurysms.

Description and clinical relevance of the variable conformation of canine spinal arachnoid diverticula.

The conformation of spinal arachnoid diverticula (SAD) and their clinical implications are poorly characterized in dogs. This retrospective cross-sectional study describes different SAD conformations in dogs and aims to identify if there is an association between SAD conformation and clinical features, localization, syringomyelia (SM) presence, concurrent vertebral condition, treatment option, and short as well as long-term outcome. Sixty-two dogs were included (12 cervical and 50 thoracolumbar SAD). All dogs with a cervical SAD had a cranial tethered conformation and were not included in the statistical analysis. Half of the dogs with a thoracolumbar SAD were cranial tethered, and the other half were caudal tethered. SM associated with SAD had a moderate prevalence in the cervical region (58.3%) and a high prevalence in the thoracolumbar region (82%). All dogs with the presence of SM and caudal tethered SAD had a cranial positioned SM, and all dogs with SM and a cranial tethered SAD had a caudal positioned SM. The SM absolute length and SM length/L2 ratio were significantly higher (P = .018, respectively) in the caudal tethered SAD compared with the cranial tethered SAD. The short-term outcome was statistically different (P = .045) between caudal and cranial tethered thoracolumbar SAD, but not the long-term outcome (P = .062). Multivariable logistic regression identified thoracolumbar caudal tethered SAD conformation had a better short-term outcome (P = 0.017, OR: 0.043, CI: 0.003-0.563), independently of SM length measurements. SAD conformation in dogs can influence SM formation. A possible link between short-term outcome and SAD conformation was found, but further research is warranted.

Endovascular approach to posterior spinal cord AV shunts via the anterior spinal artery.

Intradural spinal cord arteriovenous shunts are challenging vascular lesions with poor prognosis if left untreated. Therapeutic options include endovascular treatment, microsurgery or a combined approach. Surgical approaches are more complex if the lesions are located anteriorly and supplied by the anterior spinal artery (ASA). ASA can also vascularize shunts located on the posterior surface of the spinal cord either by transmedullary arteries, pial circumferential arteries or, if affecting the lower portions of the cord, by the anastomotic channels of the basket. Each of these vessels can be used for endovascular navigation to reach the shunts with good results if appropriate anatomical rules are followed. We describe here some technical considerations based on the anatomical analysis for the embolization of posterior spinal cord arteriovenous shunts vascularized by the anterior spinal artery.

Does Early Mobilization Following Resection of Spinal Intra-Dural Pathology Increase the Risk of Cerebrospinal Fluid Leaks?-A Dual-Center Comparative Effectiveness Research.

Background and Objectives: Prolonged bed rest after the resection of spinal intradural tumors is postulated to mitigate the development of cerebrospinal fluid leaks (CSFLs), which is one of the feared postoperative complications. Nonetheless, the empirical evidence supporting this conjecture remains limited and requires further investigation. The goal of the study was to investigate whether prolonged bed rest lowers the risk of CSFL after the resection of spinal intradural tumors. The primary outcome was the rate of CSFL in each cohort. Materials and Methods: To validate this hypothesis, we conducted a comparative effectiveness research (CER) study at two distinct academic neurosurgical centers, wherein diverse postoperative treatment protocols were employed. Specifically, one center adopted a prolonged bed rest regimen lasting for three days, while the other implemented early postoperative mobilization. For statistical analysis, case-control matching was performed. Results: Out of an overall 451 cases, we matched 101 patients from each center. We analyzed clinical records and images from each case. In the bed rest center, two patients developed a CSFL (n = 2, 1.98%) compared to four patients (n = 4, 3.96%) in the early mobilization center (p = 0.683). Accordingly, CSFL development was not associated with early mobilization (OR 2.041, 95% CI 0.365-11.403; p = 0.416). Univariate and multivariate analysis identified expansion duraplasty as an independent risk factor for CSFL (OR 60.33, 95% CI: 0.015-0.447; p < 0.001). Conclusions: In this CER, we demonstrate that early mobilization following the resection of spinal intradural tumors does not confer an increased risk of the development of CSFL.

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

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

Spinal Intradural Extramedullary Tumors: A Retrospective Analysis on Ten-Years' Experience of Minimally Invasive Surgery and a Comparison with the Open Approach.

Spinal intradural extramedullary (ID-EM) tumors are pathologies widely treated through a classical open approach. However, minimally invasive surgery is gaining traction as a comparable treatment option because it carries less morbidity and may reduce healthcare costs.This study aimed to compare the clinical and functional outcomes of open versus minimally invasive approaches for patients with ID-EM tumors. We performed a retrospective analysis on prospectively collected data from patients with ID-EM tumors submitted to surgery. Baseline features and operative variables were compared, including surgery duration and estimated blood loss (EBL). Postoperative data covered tumor histology, length of stay (LOS), complication(s), and neurological status (Medical Research Council (MRC) scale) at the last follow-up.In total, 46 patients were included: 30 (65.2%) operated through an open approach and 16 (34.8%) through a minimally invasive surgical (MIS) approach. The predominant histology type was schwannomas (43.5%). Lesions more frequently affected the lumbar spine (34.8%). The tumor dimensions were similar in both cohorts. The minimally invasive approach was on average 76.7 min faster and correlated positively with less EBL (140 mL less than that of the open approach). Patients in the MIS group had shorter LOSs (5.63 days vs. 17.27 days) and had fewer postoperative complications. No significant difference in functional outcome was found.MIS is as effective as the traditional approach in achieving comparable functional outcomes, with advantages such as shorter surgery durations, less blood loss, and shorter hospital LOSs.

Metastatic intradural primary spinal osteosarcoma: illustrative case.

BACKGROUND: Osteosarcomas are a common primary bone neoplasm among adolescents but represent 0.2% of all malignancies with an incidence of two to four cases per million persons annually worldwide. Although known to have significant metastatic potential, its rare incidence, treatment resistance, and poor prognosis have rendered it a poorly understood and infrequently documented pathology. OBSERVATIONS: Herein the authors present the first documented case of lumbosacral intradural metastasis of a primary osteosarcoma in a young patient, possibly via intradural dissemination following pinhole durotomy in a prior thoracic surgery. LESSONS: Osteosarcomas remain a difficult pathology to treat, particularly upon metastatic dissemination. The utility of adjuvant radiotherapy after resection of an osteosarcoma is increasingly evident in the reduction of local recurrence. In the context of intraoperative pinhole durotomies in resections of high-grade lesions, due consideration should be given to whole-spine radiation, although this remains an evidence-free zone.

Primary intradural Extraosseous Ewing's sarcoma of the cauda equina: A case report and literature review.

Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1. The patient underwent partial removal of the intradural lesion. Histopathological examination showed the presence of small round cells, which were consistent with Ewing Sarcoma. The patient was then treated with targeted radiation therapy and chemotherapy. The rarity of IEES in this specific location underscores the significance of evaluating and managing patients with intradural spinal tumors with careful consideration of this diagnosis. To further investigate this condition, we conducted a thorough review of the literature on IEES involving the lumbar spine and cauda equina. Our analysis revealed that patients with this condition frequently exhibit rapidly progressive neurological symptoms likely attributed to hemorrhagic transformation. This characteristic may serve as a distinguishing factor from other lesion types, particularly benign ones. Our study provides a comprehensive summary that can offer direction for clinical management in comparable uncommon and novel cases.

Spinal intradural microsurgery in a nascent neurosurgical department: Lessons learned from the first 25 cases.

BACKGROUND: Between 2017 and 2021, the newly established Department of Neurosurgery at Shaare Zedek Medical Center in Jerusalem, a high volume metropolitan hospital, operated on 25 intradural lesions in 24 patients (one patient had multiple tumors). In this retrospective study, we review results and lessons learned as experienced surgeons opened a new service line. METHODS: A multidisciplinary team was assembled and led by experienced neurosurgeons with skills in both microneurosurgery and complex spine care. Standard operative techniques were used. A chart review was done to assess complications and outcome. RESULTS: 25 lesions were reviewed in 24 patients (14 female; 10 male) between the ages of 11-82 years of age. In 14 cases, gross total resection (GTR) was achieved; 11 cases underwent partial resection. Of the 11 non-GTR cases, 3 were initially planned as biopsies. In one case, there was a significant neurologic decline directly related to surgery. In a separate case, there was iatrogenic instability, necessitating further treatment. CONCLUSIONS: We identify six lessons learned in a nascent neurosurgical department, noting that surgical excellence is of paramount importance, but that the surgeon must also expand his/her role from master technician to team leader. Both microsurgical expertise for neural anatomy and understanding of spinal biomechanics for osseous anatomy is mandatory for surgery of SIDT. This retrospective analysis of our case series demonstrates experienced neurosurgeons can successfully deploy a new service line for challenging cases to the benefit of the hospital and local community.

Spinal intramedullary schwannoma: Report of two cases with review of the literature.

INTRODUCTION: Spinal tumors comprise 15 % of all central nervous system tumors, with schwannomas accounting for 30 % of primary intraspinal neoplasms. While predominantly extramedullary-intradural, spinal schwannomas rarely manifest intramedullary occurrences (0.3 % of intraspinal tumors). This study sheds light on two rare cases of thoracic intramedullary schwannomas, emphasizing their diagnostic complexities and surgical management, alongside a literature review. CASE PRESENTATION: Case 1 involves a 50-year-old female presenting with worsening back pain, right lower limb weakness, and urinary incontinence. MRI revealed an intradural intramedullary soft tissue mass, diagnosed as a schwannoma with an associated organizing hematoma. Surgical removal led to gradual improvement. Case 2 features a 25-year-old male with back pain, partial foot drop, and weakness in the right knee and hip. MRI demonstrated an intradural intramedullary lesion, later confirmed as an intradural intramedullary schwannoma. Surgery resulted in a smooth recovery without adverse effects. DISCUSSION: This article presents two cases of intradural intramedullary thoracic schwannomas initially misdiagnosed as astrocytomas. Surgical resection confirmed the diagnosis, underscoring challenges in preoperative MRI diagnosis. The review of 174 reported cases reveals an equal distribution between the cervical and thoracic regions, with males affected 1.5 times more frequently than females. The average age of onset is 40, and surgical treatment demonstrates a 90 % improvement rate. The complex pathogenesis encompasses six proposed explanations. Clinical suspicion, considering pain and neurological symptoms, is paramount due to potential misdiagnosis and the imperative for histological confirmation. CONCLUSION: Although rare, intramedullary schwannomas (IMS) have significant clinical implications, necessitating precise treatment. Surgical resection yields favorable outcomes, with subtotal resection considered based on adhesion factors. Pre-surgical diagnosis requires a comprehensive integration of radiological and clinical data, with intraoperative analysis ensuring optimal treatment strategies.

Spinal intradural pseudocyst formation in central nervous system superficial siderosis.

The figure shows tissue samples taken from three previous cases, revealing the cause of hemosiderin deposition in the central nervous system because of superficial siderosis.

Isolated thoracic intradural extramedullary epidermoid cyst: A technical note.

Background: Congenital, acquired, and iatrogenic spinal epidermoid cysts (EC) are very rare. Methods: A 62-year-old female patient presented with a 5-month history of progressive paraparesis leading to paraplegia secondary to a posterior compressive intradural extramedullary lesion at the T7 level. The patient underwent a laminectomy/durotomy for gross total tumor excision. Results: Histopathology confirmed the lesion was an epidermoid cyst. Although her spasticity improved within 5 weeks, she only regained partial lower extremity motion (i.e., 3/5 motor function). Conclusion: Patients presenting with the acute/subacute onset of paraparesis secondary to spinal EC should undergo timely gross total cyst resections to optimize neurological outcomes.

A Rare Case of Primary Malignant Melanoma of Cervical Spine Having Extramedullary Intradural Origin: A Single Case Report and Literature Review.

Introduction: Primary spinal malignant melanoma (PSMM) of extramedullary intradural origin is a rare malignant condition with limited current literature regarding its clinical course, magnetic resonance imaging (MRI) findings, treatment strategies, and outcomes. Case Discussion: This is a case report of a patient with PSMM who was treated with surgery followed by radiotherapy for his residual disease in Shaukat Khanum Memorial Trust, Pakistan. The clinical and radiological findings of this case were retrospectively analyzed using the Hospital Information System. Practical implementations: PSMM of extramedullary intradural origin is a rare malignant tumor that shows characteristic findings on MRI. Surgical resection is the preferred treatment, and radiotherapy is useful for residual disease.

Spinal intradural extramedullary cavernoma: A case report.

INTRODUCTION: Cavernomas are rare vascular lesions that can occur anywhere along the neuraxis. However, they are most commonly found in the cerebral hemispheres. Spinal cavernomas are more uncommon and intradural extramedullary cavernomas are the most uncommon as they constitute only 3 % of spinal cavernomas. PRESENTATION: A 36-year-old female presented to our neurosurgical clinic with a history of back pain radiating to the left side of the chest with left lower extremity paresthesia and ataxia without urinary disturbance. Neurological exam showed left-sided hypoesthesia below the T9 dermatome in addition to increased patellar and Achilles reflexes on the left side. MRI showed a homogeneous intradural extramedullary mass which was hyperintense on T1 and hypointense on T2 and it was surgically resected. Pathological examination confirmed the diagnosis of intradural extramedullary cavernoma. DISCUSSION: Intradural extramedullary cavernomas are extremely rare lesions that arise within the area located between the inner surface of the dura and the pial surface of the spinal cord. Up until 2022 only 40 cases of intradural extramedullary cavernomas were reported in the literature. MRI is the preferred imaging modality and pathology is the golden standard for diagnosis. Surgical resection showed very promising results and it is considered the golden standard for treating this condition. However, surgery should be performed urgently to give an optimal outcome. CONCLUSION: Clinicians should consider this condition in their differential diagnoses when faced with progressive spinal root compression symptoms, sudden onset myelopathy, or progressive subarachnoid hemorrhage.

Characteristics and Outcome of Surgically Treated Patients with Intradural Extra- and Intramedullary Spinal Metastasis-A Single-Center Retrospective Case Series and Review.

OBJECTIVE: Intradural spinal metastases are considered rare. At present, limited information is available on incidence, surgical management, and outcomes. METHODS: We conducted a retrospective patient chart review from 2002 to 2024, identifying all patients surgically treated for intradural spinal metastases. Clinical, surgical and survival data were collected and compared to literature data for patients surgically treated for extradural spinal metastases. RESULTS: A total of 172 patients with spinal metastases were identified with 13 patients meeting inclusion criteria (7.6%). The mean age at diagnosis of intradural spinal metastases was 52 ± 22 years, with diverse primaries including lung (n = 3), breast (n = 2), sarcoma (n = 2), and six unique entities. Intradural spinal metastasis was diagnosed on average of 3.3 years after primary diagnosis. In total, we observed five (38%) intradural-extramedullary and eight (62%) intramedullary metastases, located in the cervical (38.5%), thoracic (46.1%) and lumbar spine (15.4%). The most common preoperative symptoms were pain, sensory changes, and gait ataxia (each 76.9%). Gross total resection was achieved in 54%, and local tumor control in 85%. Postoperatively, 92% exhibited clinical improvement or stability. Most frequent adjuvant treatment was radio- and/or chemotherapy in 85%. The average survival after operation for spinal intradural metastases was 5 months, ranging from 1 month to 120 months. The location of the intradural metastasis in the cervical spine was associated with a significantly more favorable survival outcome (compared to thoracic/lumbar location, p = 0.02). CONCLUSIONS: Intradural location of spinal metastases is rare (7.6%). Even so, surgical resection is safe and effective for neurological improvement, and survival appears lower compared to the reported survival of extradural spinal metastases.

Thoracic root-related intradural extramedullary cavernoma presenting with subarachnoid hemorrhage: illustrative case.

BACKGROUND: Just 5% of all cavernomas are located in the spine. Thoracic root-related subtypes are the rarest, with a total of 14 cases reported in the literature to date. Among them, only 4 presented with subarachnoid hemorrhage (SAH). OBSERVATIONS: A 65-year-old female presented after an ictus of headache with no neurological deficits. Computed tomography (CT) demonstrated sulcal SAH, with the remainder of the workup nondiagnostic for etiology. Three weeks later, she re-presented with acute thoracic back pain and thoracic myelopathy. CT and magnetic resonance imaging suggested dubiously a T9-10 disc herniation with spinal cord compression. Surgical decompression and resection were then planned. Intraoperative ultrasound (IUS) demonstrated an intradural extramedullary lesion, confirmed to be cavernoma. Complete resection was achieved, and the patient was discharged a few days postoperatively to inpatient rehabilitation. LESSONS: Although spine imaging is deemed to be low yield in the evaluation of cryptogenic SAH, algorithms can be revisited even in the absence of spine-related symptoms. Surgeons can be prepared to change the initial surgical plan, especially when preoperative imaging is unclear. IUS is a powerful tool to assess the thecal sac after its exposure and to help guide this decision, as in this rare entity.

Sacral nerve root metastasis in a patient with lung carcinoma resembling neurinoma - a case report and literature review.

Intradural extramedullary metastases from systemic neoplasms are very rare, with an incidence ranging from 2% to 5% of all secondary spinal diseases. We present the case of a 53-year-old man diagnosed with lung adenocarcinoma with symptoms of severe back pain and tibial paresis. The magnetic resonance imaging (MRI) revealed an intradural lesion originating from the right S1 nerve root mimicking neurinoma. Total tumor removal was achieved via posterior midline approach. The histological examination was consistent with lung carcinoma metastasis. Due to the rarity of single nodular nerve root metastases, MRI images may be misinterpreted as nerve sheath tumors, such as schwannomas or neurofibromas. We performed a brief literature review outlining the mainstay of diagnosis, therapeutic approach, and the prognosis of these rare lesions.

A rare case of multifocal craniospinal leptomeningeal melanocytoma: A case report and scoping review.

Introduction: Leptomeningeal melanocytomas are rare tumours originating from neural crest derived melanocytes. They are usually solitary and presentation with multifocal meningeal melanocytoma is very rare and indicative of potentially more aggressive behaviour. This case report and scoping review sought to evaluate the presentation, and key radiological features that can help differentiate multifocal meningeal melanocytoma from other differentials and provide a discussion of the key management and prognostic points once these tumours are diagnosed. Case presentation: A 26 year old male presented with neck pain radiating to both shoulders and subjective weakness in left shoulder movement. MRI demonstrated a large enhancing C2-C3 intradural-extramedullary lesion with further lesions at the T7/T8 level, left cerebellopontine angle and midline suprachiasmatic region. Whilst the imaging appearances were initially thought be indicative of a phacomatosis such as NF2-related schwannomatosis, surgical excision of the cervical tumour confirmed a melanocytic tumour of leptomeningeal origin, consistent with multifocal meningeal melanocytoma. Patient made a good post-operative recovery and remains under half yearly radiological surveillance, with repeat MRI 6 months after surgery demonstrating subtle growth of the untreated intracranial and spinal lesions. Literature review and conclusions: This is the first description, to our knowledge, of a multifocal meningeal melanocytoma associated with both cerebellopontine angle and suprasellar lesions. This case and included scoping review highlight the need to consider this rare diagnosis whenever multifocal craniospinal lesions are encountered, and the need to consider aggressive management through surgical resection and adjuvant craniospinal radiotherapy once these tumours are diagnosed.