Understanding metastatic involvement of the conus medullaris: a systematic review of clinical presentations, diagnostic approaches, treatment options, and patient outcomes.

INTRODUCTION: Metastasis to the conus medullaris (CM) is a rare but devastating condition. This systematic review aimed to evaluate the clinical presentation, diagnostic workup, treatment options, and outcomes of patients with CM metastasis. By synthesizing the available evidence, this study seeks to improve our understanding of this condition and inform clinical practice. MATERIALS AND METHODS: A systematic review adhering to PRISMA guidelines analyzed literature on CM metastasis from 1997 to January 2024. Human studies in English were included, focusing on primary research articles. Screening criteria ensured a homogeneous study population, with data analyzed using SPSS 26 and assessed for quality using the JBI checklist. RESULTS: The study analyzed 88 patients with conus medullaris metastasis. Common symptoms included back pain (49.3%), sensory impairment (75%), and bladder dysfunction (60.3%). MRI was the primary diagnostic tool, revealing lesions above L1 (37%) or between L1 and L2 (29%). Treatment involved surgery with laminectomy, and combined therapy (surgery plus radiotherapy) in 81.3%. Postoperative outcomes showed improved motor function in 59.6% of patients, while combined therapy yielded better sensory and bowel/bladder function recovery. Median survival was 100 days. CONCLUSION: Metastasis to the conus medullaris is rare but significant. Surgical resection can improve motor function, while combined therapy (surgery plus radiotherapy) is effective in improving sensory manifestations and bowel/bladder functions. Despite these treatments, the median survival remains around 100 days, which is shorter compared to other types of intramedullary spinal cord metastases.

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

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

A case report of spinal toxocariasis with extensive tumor-like involvement.

BACKGROUND: Toxocariasis is a common parasitic infection worldwide. Although it can present as several clinical syndromes, neurological manifestation is rare. Only a few reports are available on spinal cord involvement of toxocariasis. We report a case that presented with gait disturbance due to progressive lower limb spasticity. The patient had had visceral toxocariasis infection 8 years before. A spine magnetic resonance image (MRI) showed syringomyelia along the entire thoracic cord with small nodular enhancing lesions in the mid-portion of the syrinx, which led to the suspicion of ependymoma. Surgical mass removal was performed. However, histopathological examination of the mass did not show any malignant cells; instead, there were numerous axonal retraction balls with an eosinophilic granular body-like appearance. The serum antibody titer against toxocariasis was borderline high. Taken together, these observations led to a diagnosis of Toxocara infection, and the patient was treated with albendazole. CONCLUSION: To the best of our knowledge, this is the first case report of tumor-like spinal toxocariasis involving extensive lesions. A solid enhancing mass with accompanied syrinx and hemorrhage might be a Toxocara infection. It can easily be diagnosed with serologic tests and simply be treated with oral albendazole if suspected.

Predictors for spinal deformity following resection of intramedullary tumor via posterior approach: a systematic review and meta-analysis.

PURPOSE: The present study aimed to identify the clinical predictive factors for worsened spinal deformity (SD) following surgical resection via posterior approach for primary intramedullary tumors. METHODS: A systematic search was performed using PubMed, Web of Science, and Scopus databases to extract potential references. Observational studies reporting predictive factors for worsened SD following surgical resection via posterior approach for primary intramedullary tumors were included. The odds ratio (OR) was calculated for dichotomous parameters. RESULTS: Four retrospective cohort studies were included in the meta-analysis. They were comprised of two groups of patients; those who developed SD (n = 87) and those who did not (n = 227). For patients with IMSCTs, age under 25 years as well as age under 13 years were the demographic variables associated with postoperative SD (odds ratio [OR] 3.92; p = 0.0002 and OR 4.22; p = 0.003). In both the fusion and the non-fusion subgroups, preoperative spinal deformity strongly predicted postoperative SD (OR 11.94; p < 0.001), with the risk highly elevated among the non-fusion patients (OR 24.64; p < 0.0002). Thoracolumbar junction involvement was also found to be a predictor of postoperative SD for patients with IMSCT (OR 2.89; p = 0.02). CONCLUSION: This study highlights the importance of considering age, preoperative spinal deformity, and thoracolumbar junction involvement as predictors of postoperative spinal deformity following surgical resection for IMSCT. These findings may provide guidance for the management of these patients, including the development of preoperative planning strategies and the selection of the most appropriate surgical approach for high-risk patients.

Psychological and Psychiatric Aspects of Brain and Spinal Cord Tumors.

Central nervous system (CNS) tumors are mainly diagnosed by physical symptoms such as paralysis, visual field defect, seizure, and loss of consciousness. The psychological and psychiatric background of CNS tumors, whether in preoperative or postoperative period, has long been a neglected topic; however, lately, many authors and researchers have paid more attention to these manifestations. Neurocognition is a subset of parameters, including attention, memory, mood, emotions, language production, personality, executive function, problem-solving, calculation, and spatial cognition, making up the patient's cognitive performance. Also, it is worthy to say that neurocognition is considered a parameter of quality of life (QoL). Currently, we know that neurocognitive disorders are a group of symptoms presenting by the patients. These symptoms may be the first picture of CNS lesions, which result in incorrect treatment, a higher financial burden on the patient and health system, and finally, poorer QoL and performance scale if they are not diagnosed early. Psychological and psychiatric problems such as depression, anxiety, and phobia following the CNS tumors have two aspects. These may present before any treatment resulting from the tumoral mass effect, peritumoral edema, or cerebral tissue disruption due to the space-occupying lesion. On the other hand, we can see these features after a kind of therapy such as surgery, medical therapy, or adjuvant therapy. Sometimes, the CNS tumors lead to psychosocial complications postoperatively. Indeed, considering tumor surgery complications, some patients may find various degrees of deficits that make the patient isolated either socially or professionally. Obviously, the improvement rate and outcome of this specific situation depend on the mechanism of occurrence and its causes. For instance, postoperative symptom relief would be expected when the symptoms are related to the tumoral mass effect. Getting familiar with this constellation of the symptoms, realizing them, and then localizing them to the correct area of the CNS are very crucial. Accordingly, because of their importance in QoL, their influence on patient's survival even more than the extent of resection of the tumor, and somehow their ignorance, we will discuss different neurocognitive manifestations related to CNS tumors in this chapter.

The Role of Nanotechnology in Spinal Cord Tumors.

The efficacy of current multimodal therapeutic strategies in spinal cord tumors is limited by the lack of specific therapies. Importantly, sufficient amount of therapeutic materials should be concentrated in tumors in order to be efficient. Overcoming the blood-brain barrier is the major obstacle for chemotherapeutics, which cannot reach the tumor bed in efficacious doses. The intrinsic properties of nanoparticles make them suitable for activating numerous processes both at the cellular and subcellular levels, making them good candidates to be used for different purposes in medicine. Furthermore, the adaptability characteristic of NPs may enable them to pass through the blood-brain barrier and transport different pharmacological compounds. Nanoparticle systems provide prolonged drug delivery directly to the tumor or by functionalizing the material surface with peptides and ligands allowing the drug-loaded material to specifically target the tumor cells. In this chapter, various preclinical and/or clinical studies in treatment of spinal cord tumors are discussed.

Malignant Spinal Tumors.

Malignant spinal tumors constitute around 22% of all primary spinal tumors. The most common location of metastases to the spinal region is the extradural compartment. The molecular and genetic characterization of these tumors was the basis for the updated WHO classification of CNS tumors in 2016, where many CNS tumors are now diagnosed according to their genetic profile rather than relying solely on the histopathological appearance. Magnetic resonance imaging (MRI) is the current gold standard for the initial evaluation and subsequent follow-up on intradural spinal cord tumors, and the imaging sequences must include T2-weighted images (WI), short time inversion recovery (STIR), and pre- and post-contrast T1-WI in the axial, sagittal, and coronal planes. The clinical presentation is highly variable and depends on the tumor size, growth rate, type, infiltrative, necrotic and hemorrhagic potential as well as the exact location within the spinal compartment. Surgical intervention remains the mainstay of management of symptomatic and radiographically enlarging spinal tumors, where the goal is to achieve maximal safe resection. Tumor recurrences are managed with repeat surgical resection (preferred whenever possible and safe), radiotherapy, chemotherapy, or any combination of these therapies.

Posterolateral transpedicular approach for ventrally seated cervicothoracic spinal cord lesions: how I do it.

BACKGROUND: Surgical exposure of lower cervical and upper thoracic intradural extramedullary lesions located along the ventral medulla are among the most complexes to address in spinal surgery, and their surgical removal carries a high risk. METHODS: We describe the surgical steps of a posterolateral transpedicular approach for resection of an intradural extramedullary lesion located anterolaterally at C7-T1 level. CONCLUSIONS: A posterolateral transpedicular approach is a safe and efficient surgical corridor to explore the ventral spinal cord and to have a direct access to lower cervical-upper thoracic lesions without the extensive manipulation of the spinal cord and the spine instability.

Feasibility of multimodal intraoperative neurophysiological monitoring for extramedullary spinal cord tumor surgery in elderly patients.

BACKGROUND/PURPOSE: Extramedullary spinal cord tumors (EMSCTs) are mostly benign tumors which are increasingly diagnosed and operatively treated in the elderly. While there are hints that multimodal intraoperative neurophysiological monitoring (IONM) could be influenced by age and age-related comorbidities, no study has ever systematically evaluated its feasibility and value for EMSCT surgery in elderly patients. METHODS: We retrospectively evaluated all patients with microsurgical EMSCT resection under continuous multimodal IONM with SSEPs, MEPs and electromyography between 2016 and 2020. Epidemiological, clinical, imaging and operative/IONM records as well as detailed individual outcomes were analyzed and compared for the cohort < / ≥ 65 years. RESULTS: Mean age was 45 years in cohort < 65 years (n = 109) and 76 years in cohort ≥ 65 years (n = 64), while baseline/operative characteristics did not significantly differ. Mean baseline SSEPs' latencies (left-right average) were significantly higher in the cohort ≥ 65 years for both median (20.9 ms vs. 22.1 ms; p < 0.01) and tibial nerve (42.9 ms vs. 46.1 ms; p < 0.01) without significant differences for SSEPs' amplitudes. Stimulation intensity to elicit intraoperative MEPs was significantly higher in the cohort ≥ 65 years (surrogate-marker: left-right-averaged quotient ID1-muscle/abductor-hallucis-muscle; 1.6 vs. 2.1; p < 0.001). Intraoperatively, SSEP and MEP monitoring were feasible in 99%/100% and 99%/98% for the cohort < / ≥ 65 years without significant differences in rates for significant IONM changes during surgery or postoperatively new sensorimotor deficits. Sensitivity of IONM was 29%/43%, specificity 99%/98%, positive and negative predictive values 67%/75% and 95%/93% for the cohort < / ≥ 65 years. Overall, age was no risk factor for IONM feasibility or rate of significant IONM changes. DISCUSSION: Multimodal IONM is feasible/reliable for EMSCT surgery in elderly patients. An age-related prolongation of SSEPs' latencies and demand for higher stimulation intensities for MEPs' elicitation has to be considered.

Advances in Imaging Modalities for Pediatric Brain and Spinal Cord Tumors.

BACKGROUND: Neuroimaging has evolved from anatomical imaging toward a multi-modality comprehensive anatomical and functional imaging in the past decades, important functional data like perfusion-weighted imaging, permeability imaging, diffusion-weighted imaging (DWI), and diffusion tensor imaging (DTI), tractography, metabolic imaging, connectomics, event-related functional imaging, resting state functional imaging, and much more is now being offered. SUMMARY: Precision diagnostics has proven to be essential for precision treatment. Many minimal invasive techniques have been developed, taking advantage of digital subtraction angiography and interventional neuroradiology. Furthermore, intraoperative CT and/or MRI and more recently MR-guided focused ultrasound have complemented the diagnostic and therapeutic armamentarium. KEY MESSAGES: In the current manuscript, we discuss standard imaging sequences including advanced techniques like DWI, DTI, susceptibility-weighted imaging, and 1H magnetic resonance spectroscopy, various perfusion weighted imaging approaches including arterial spin labeling, dynamic contrast enhanced imaging, and dynamic susceptibility contrast imaging. Pre-, intra, and postoperative surgical imaging including visualize imaging will be discussed. The value of connectomics will be presented for its value in neuro-oncology. Minimal invasive therapeutic possibilities of interventional neuroradiology and image-guided laser ablation and MR-guided high-intensity-focused ultrasound will be presented for treatment of pediatric brain and spinal cord tumors. Finally, a comprehensive review of spinal cord tumors and matching neuropathology has been included.

Approaches to Incidental Intradural Tumors of the Spine in the Pediatric Population.

BACKGROUND: Incidental intradural tumors of the spine in the pediatric population are rare lesions whose management remains unclear. Surgeons must balance the risks of iatrogenic deficits and complications after surgical resection against the risks from progressive growth of the tumor. Moreover, the natural history of an incidental finding can be difficult to predict. Here, we review the literature on incidental intradural tumors of the spine and present considerations for their management. SUMMARY: Growth of the tumor or changes in radiographic features are usually indications for resection. Asymptomatic lesions can be found in patients with genetic syndromes that predispose to tumor formation, such as neurofibromatosis type 1 and 2, schwannomatosis, and Von-Hippel-Lindau syndrome, and careful workup of a genetic cause is warranted in any patient presenting with multiple tumors and/or cutaneous features. Close follow-up is generally favored given the heavy tumor burden; however, some recommend pre-emptive resection to prevent permanent neurological deficits. Incidental intradural tumors can also occur in association with hydrocephalus, significant syringomyelia, and cord compression, and surgical treatment is usually warranted. Tumors may also be discovered as part of the workup for scoliosis, where they are not truly incidental to the scoliosis but rather are contributing to curve deformation. KEY MESSAGES: Thorough workup of patients for associated genetic syndromes or comorbidities should be undertaken in pediatric patients with incidental intradural tumors. Further research is needed into the natural history of these incidental lesions. Incidental tumors can often be managed conservatively with close follow-up, with surgical intervention warranted for expanding tumors or new-onset symptoms.

Collision tumor between a spinal schwannoma and chronic lymphocytic leukemia/small lymphocytic lymphoma: case report and review of the literature.

A collision tumor is one where two neoplasms of differing type occur at the same anatomical site. We present a patient suffering from non Hodgkin small cell lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) and complaining intense lumbar back pain refractory to medical treatment. Lumbosacral MRI showed an intradural extramedullary lesion in the left L2-L3 foramen with extracanalar development and compression of psoas muscle. CT showed intralesional calcification. The patient underwent resection of the lesion through a paraspinal posterolateral approach (Wiltse approach). The histology was of schwannoma with intralesional calcifications and lymphocitic infiltrates compatible with B-lineage SLL/CLL. After the operation the patient suffer from left psoas muscle motor weakness (3/5 MRC). Because of hematological disease progression, she underwent 6 cycles of chemioterapy (Fludarabine, Cyclophosphamide, Rituximab). At a six-month follow-up no recurrence or residual tumor upon lumbosacral MR imaging was detectable and the left thigh flexion returned normal. To our knowledge, this is the first described case in the literature of collision tumor between a solitary spinal Schwannoma and SLL/CLL.

Non-dysraphic extramedullary intradural spinal lipoma with neurocutaneous melanocytosis.

INTRODUCTION: Intradural spinal lipomas are very rare and constitute less than 1% of all spinal tumors. Such tumors are usually associated with spinal dysraphism and occur mostly in the lumbosacral or cervical region. Intradural spinal lipomas tends to be intramedullary or subpial. Meningeal melanocytoma is further rarer cases that comprise less than 0.1% of cases. These usually occur in the fifth or fifth decade and chances of malignant transformation are high. CASE REPORT: Here, we report an extremely rare case (first to the best of our knowledge) of a 9 years female child who presented to us with rapid progressing paraparesis. She was operated and found to have an intradural purely extramedullary spinal lipoma without spinal dysraphism. Moreover, she had melanin pigment deposits all over her meninges which is further rare. On presentation, the patient was bedridden but after surgery, the patient improved and could walk without support. CONCLUSIONS: To the best of our knowledge, this is the first case of spinal cord lipoma in dorsal location along with melanin pigments in the meninges. We discuss the pathogenesis, presentation and management of intradural extramedullary spinal lipomas.

Usefulness and limitations of intraoperative pathological diagnosis using frozen sections for spinal cord tumors.

BACKGROUND: Intraoperative pathological diagnosis has a major influence on the intra- and postoperative management of spinal cord tumors. Thus, the aim of this study was to assess the reliability of intraoperative pathological diagnosis for spinal cord lesions by comparing it with the final pathological diagnosis and to determine its usefulness and limitations. METHOD: Three-hundred and three consecutive patients (mean age, 53.9 years) with neoplastic spinal cord lesions who underwent initial surgery between 2000 and 2021 were included. The anatomical locations of the spinal cord tumors and the implementation rate of intraoperative pathological diagnosis in each tumor type were evaluated. As the primary outcome, we determined the concordance rates between the intraoperative pathological diagnosis and the final diagnosis. When the intraoperative pathological diagnosis and final diagnosis were the same, the diagnosis was defined as a "match." Otherwise, the diagnosis was defined as a "mismatch." RESULTS: The overall implementation rate of intraoperative pathological diagnosis was 53%, with implementation rates of 71%, 45%, 47%, and 50% for intramedullary, intradural extramedullary, extradural, and dumbbell tumors, respectively. The overall concordance rate was 87.6%, with concordance rates of 80%, 95%, 75%, and 90% for intramedullary, intradural extramedullary, extradural, and dumbbell tumors, respectively (p < 0.05). The diagnoses of ependymomas, low-grade astrocytomas, and high-grade astrocytomas was occasionally difficult among intramedullary tumors. Among intradural extramedullary tumors, differentiation between grade 1 meningioma and high-grade meningioma was difficult using intraoperative pathological diagnosis. CONCLUSIONS: Surgeons must recognize the lower accuracy of intraoperative pathological diagnosis for intramedullary and extradural lesions and make a final decision by considering the intraoperative gross findings, preoperative clinical course, and imaging.

Perioperative lumbar subarachnoid drainage could not prevent postoperative CSF leakage after spinal cord tumor resection using an artificial dura mater.

BACKGROUND: Cerebrospinal fluid (CSF) leakage occurs in patients who undergo dural repair using artificial dura mater. This study aimed to determine if perioperative lumbar subarachnoid drainage could reduce the incidence of postoperative CSF leakage in cases of dural repair using artificial dura mater. METHODS: We retrospectively analyzed 84 patients (41 men, 43 women; mean age, 52.2 ± 20.1 years) who underwent intradural spinal cord tumor resection and dural repair using artificial dura mater. These patients were divided according to whether they underwent perioperative lumbar subarachnoid drainage (39 patients: D group) or had no drainage (45 patients: ND group). The incidence of radiographic and symptomatic CSF leakage as well as baseline characteristics and operative data were compared between the two groups. RESULTS: Radiographic CSF leakage was observed in 21 patients (25.0%), including 10 (25.6%) in the D group and 11 (24.4%) in the ND group. Symptomatic CSF leakage was observed in 12 patients (14.2%), including six (15,4%) in the D group and 11 (13.3%) in the ND group. There were no significant differences in the incidence of subcutaneous CSF accumulation and symptomatic CSF leakage between the two groups. In cases with symptomatic CSF leakage, the onset time of CSF leakage tended to be earlier (5.7 days vs 15.7 days), and the treatment period tended to be longer (5.8 weeks vs 2.8 weeks) in the ND group than in the D group. CONCLUSIONS: Perioperative lumbar subarachnoid drainage did not reduce the incidence of either radiographic or symptomatic CSF leakage. However, it might shorten the treatment period and reduce refractory CSF leakage, which requires multiple treatments over a long period.

Impact of tumor-associated syrinx on outcomes following resection of primary ependymomas of the spinal cord.

PURPOSE: To interrogate the association of tumor-associated syrinxes with postoperative neurological and oncological outcomes in patients surgically treated for WHO grade 2 spinal ependymomas. METHODS: Adults treated for primary spinal intramedullary ependymomas between 2000 and 2020 were identified and data were gathered on preoperative neurological exam, radiographic characteristics, operative details, and postoperative neurological outcome. Neurological status was graded on the modified McCormick Scale (MMS). Neurological worsening immediately postoperatively and at last follow-up were defined by ≥ 1 MMS grade deterioration. Decision-tree analyses were also performed to identify independent predictors of new neurological deficits. RESULTS: Seventy patients were identified; mean age 45.4 ± 12.7; 60% male. Forty-eight patients (68.6%) had tumor-associated syrinxes, were more common among males (68.8%) and cervical lesions (68.8 vs. 31.8%; P = 0.005). Postoperatively patients with syrinxes had better MMS (P = 0.035) and were less likely to require a gait aid (39.6 vs. 81.8; P = 0.002). This latter difference persisted to last follow-up (22.9 vs. 59.1%; P = 0.006). On decision-tree analysis the strongest predictors of long-term neurological worsening were advanced age (≥ 63 years) and worse baseline neurological function. Worsened neurological status in the immediate postoperative period was best predicted by thoracic localization, the presence of a hemosiderin cap, and longer craniocaudal extension. CONCLUSION: For spinal ependymomas, tumor-associated syrinxes may portend decreased risk for immediate postoperative neurologic deficits but do not predict long-term neurological outcomes (MMS) or odds of successful gross total resection. Thoracic localization appears to best predict new immediate postoperative deficits, and worse baseline neurological function and advanced age best predict long-term deficits.

Orthostatic tremor secondary to primary malignant melanoma of the spinal cord: A case report.

BACKGROUND AND PURPOSE: Orthostatic tremor and spinal cord melanoma are rare entities and seem unlikely to be associated. Herein, we report a patient diagnosed with orthostatic tremor secondary to primary malignant melanoma of the spinal cord. CASE PRESENTATION: We report the case of a 67-year-old man who experienced tremor when he was standing, which disappeared when he was sitting or walking. He also reported gait disturbance and cognitive dysfunction. Electromyography revealed a regular and symmetric high-frequency tremor in the lower extremities. The patient was admitted to a hospital several times and was diagnosed with primary orthostatic tremor and later hydrocephalus; thus, he received a ventriculoperitoneal shunt. Finally, he showed symptoms of the presence of melanoma in the spinal cord, which was supported by spinal cord magnetic resonance imaging findings. Primary malignant melanoma of the spinal cord was confirmed postoperatively. CONCLUSIONS: Orthostatic tremor is a rare entity that can be characterized by specific high-frequency tremors when the subject is standing. Considering that it remains unknown why this condition appears, some possible associations, such as primary spinal cord melanoma, should be considered. Thus, a comprehensive assessment of these types of patients is required. Our case report may facilitate the understanding of the pathophysiology and clinical symptoms of this disease.

Autonomic dysreflexia associated with cervical spinal cord gliofibroma: case report.

BACKGROUND: Autonomic dysreflexia (AD) is an abnormal reflex of the autonomic nervous system normally observed in patients with spinal cord injury from the sixth thoracic vertebra and above. AD causes various symptoms including paroxysmal hypertension due to stimulus. Here, we report a case of recurrent AD associated with cervical spinal cord tumor. CASE PRESENTATION: The patient was a 57-year-old man. Magnetic resonance imaging revealed an intramedullary lesion in the C2, C6, and high Th12 levels. During the course of treatment, sudden loss of consciousness occurred together with abnormal paroxysmal hypertension, marked facial sweating, left upward conjugate gaze deviation, ankylosis of both upper and lower extremities, and mydriasis. Seizures repeatedly occurred, with symptoms disappearing after approximately 30 min. AD associated with cervical spinal cord tumor was diagnosed. Histological examination by tumor biopsy confirmed the diagnosis of gliofibroma. Radiotherapy was performed targeting the entire brain and spinal cord. The patient died approximately 3 months after treatment was started. CONCLUSIONS: AD is rarely associated with spinal cord tumor, and this is the first case associated with cervical spinal cord gliofibroma. AD is important to recognize, since immediate and appropriate response is required.

Surgical treatment of intramedullary spinal cord metastases: functional outcome and complications-a multicenter study.

Intramedullary spinal cord metastasis (ISCM) is a rare event in the course of advanced malignancy. Management of these lesions remains controversial. Recently, surgery for ISCM has been advocated for selected patients. We performed a retrospective analysis of the clinical course, complications, and outcome of 30 patients surgically treated for ISCM. Patients' age, histopathological diagnoses of primary cancer, tumor size, spinal location, and extramedullary tumor dissemination were collected. Preoperative functional status, pre- and postoperative neurological status, and extent of the tumor resection were also analyzed. Predominant tumor location was thoracic, followed by cervical and conus medullaris. Lung cancer constituted the majority of primary malignancies. In 9 cases, one of the indications for spinal surgery was to obtain a histopathological diagnosis. On admission, all patients presented with neurological symptoms suggestive of myelopathy. After surgery, 18 patients exhibited improvement of symptoms in terms of pain relief and partial recovery of motor and/or sensory deficits; 6 patients were unchanged, while 6 patients exhibited postoperative deterioration. Median survival time after surgery was 9.9 months. Age > 70 years old, presence of systemic metastases, preoperative neurological non functional status, and lung cancer as primary tumor were all factors associated with a worse survival prognosis. This study did not show a clear survival difference between gross total and subtotal ISCM tumor resection. Patients who underwent gross total resection had a worse functional outcome with respect to patients with only partial resection. Gross total resection with low morbidity must be the surgical target, but when not possible, subtotal resection and adjuvant therapy are a valid therapeutic option.

Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population.

Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6 years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality.