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Background and Objectives: Spinal meningiomas, which are well characterized and are most frequently intradural extramedullary tumors, represent 25% of all intradural spinal tumors. The goal of this study was to compare the outcomes of surgically treated patients with spinal meningiomas in two time intervals with special emphasis on postoperative functional outcomes. Methods: Patients with spinal meningiomas admitted to our department between 1990 and 2020 were enrolled and divided into a historic cohort (HC; treated 1990−2007) and a current cohort (CC; treated 2008−2020). Patients’ clinical data and surgical and radiological reports were retrospectively analyzed up to 5 years. Preoperative and postoperative neurological function were assessed using the modified McCormick Scale (mMCS). The Charlson Comorbidity Index (CCI) was used to evaluate the effect of comorbidities on the preoperative status and postoperative outcome. Results: We included 300 patients. Participants in the CC (n = 144) were significantly younger compared to those in the HC (n = 156), with twice as many patients <50 years of age (p < 0.001). The most common tumor location was the thoracic spine (n = 204). The median follow-up was 38.1 months (±30.3 standard deviation). A symptom duration until surgery <12 months was significantly associated with an earlier improvement in the mMCS (p = 0.045). In the CC, this duration was shorter and patients’ neurological function at the first and last follow-ups was significantly better than for those in the HC (p < 0.001 for both). Conclusions: Our study results suggested that the impact of surgical management and postoperative rehabilitation on spinal meningioma patients’ long-term neurological outcome has reached important milestones over the last decades. An earlier diagnosis led to earlier surgical treatment and improved patients’ postoperative neurological recovery. Our results exposed that surgical therapy for spinal meningioma should be performed within 12 months after appearance of symptoms to achieve a better recovery.

Background: Intramedullary spinal cord tumors are rare lesions of the central nervous system. Anatomical, molecular and radiological features are well defined, but correct management is still matter of debate. Pertinent literature has reported conflicting opinions regarding the use of intraoperative electrophysiological monitoring (IONM) in the surgical treatment of this kind of lesions, recently. We report a retrospective study from two Italian centers, in order to highlight the usefulness of IONM in the management of intramedullary lesions., Methods: We performed a retrospective review of patients with intramedullary spinal tumor who underwent surgical resection from February 2011 to February 2018 in two different institutions. Clinical and radiological data, lesion features, timing of symptom onset and IONM findings were recorded. The IONM included somatosensory-evoked potentials (SSEP), motor-evoked potentials (MEP) and D-Wave whenever possible. We evaluated the outcome according to the Modified McCormick scale. We also evaluated the accuracy and relevance of surgical outcomes for each evoked potential (SSEP, MEP, D-Wave)., Results: A total of 57 patients were included. A gross total removal was achieved in 46 cases. Neurological follow-up was assessment at 3 days, and 3 and 6 months after surgery. Comparing the preoperative status and 6 months follow-up: the modified McCormick scale showed a neurological stability for 30 patients (52.63%), a worsening of neurological status for 7 patients (12.28%) and an improvement for 20 patients (35.08%). IONM presented high accuracy (sensibility of 100% and specificity of 95.65%) and significantly predicted postoperative permanent motor deficits (P<0.0001; AUC=0.978). D-Wave appeared to have significant greater predictive value than MEP and especially SSEP alone (0.967 vs 0.722 vs 0.542; P=0.044 and P<0.001 respectively)., Conclusions: The gold standard in the intramedullary lesion treatment is maximal safe resection with good neurological outcome, as shown in our patients. The use of IONM is helpful in intramedullary tumors resection in order to minimize postoperative neurological deficits and our analysis suggests that the use of D-Wave presents a statistically significant higher accuracy for predicting postoperative deficits than SSEP and MEP alone.

Intramedullary spinal cord tumors are a rare and understudied cancer with poor treatment options and prognosis. Our prior study used a combination of PDGF-B, HRAS, and p53 knockdown to induce the development of high-grade glioma in the spinal cords of minipigs. In this study, we evaluate the ability of each vector alone and combinations of vectors to produce high-grade spinal cord gliomas. Eight groups of rats ( n = 8/group) underwent thoracolumbar laminectomy and injection of lentiviral vector in the lateral white matter of the spinal cord. Each group received a different combination of lentiviral vectors expressing PDGF-B, a constitutively active HRAS mutant, or shRNA targeting p53, or a control vector. All animals were monitored once per week for clinical deficits for 98 days. Tissues were harvested and analyzed using hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining. Rats injected with PDGF-B+HRAS+sh-p53 (triple cocktail) exhibited statistically significant declines in all behavioral measures (Basso Beattie Bresnahan scoring, Tarlov scoring, weight, and survival rate) over time when compared to the control. Histologically, all groups except the control and those injected with sh-p53 displayed the development of tumors at the injection site, although there were differences in the rate of tumor growth and the histopathological features of the lesions between groups. Examination of immunohistochemistry revealed rats receiving triple cocktail displayed the largest and most significant increase in the Ki67 proliferation index and GFAP positivity than any other group. PDGF-B+HRAS also displayed a significant increase in the Ki67 proliferation index. Rats receiving PDGF-B alone and PDGF-B+ sh-p53 displayed more a significant increase in SOX2-positive staining than in any other group. We found that different vector combinations produced differing high-grade glioma models in rodents. The combination of all three vectors produced a model of high-grade glioma more efficiently and aggressively with respect to behavioral, physiological, and histological characteristics than the rest of the vector combinations. Thus, the present rat model of spinal cord glioma may potentially be used to evaluate therapeutic strategies in the future.

Radiation-induced spinal glioblastoma is an extremely rare disease with only four previously published reports in the literature. We report the fifth case, a 69-year-old woman who previously underwent treatment with brachytherapy for cervical cancer, and thereafter presented with neurologic deficits from a conus medullaris tumour. Biopsy and histopathology confirm glioblastoma, not otherwise specified. Treatment of spinal glioblastoma consists of surgery, either biopsy or excision and chemoradiation. However, results are still unsatisfactory and prognosis remains poor., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Radiation-induced spinal cord cavernous malformations (CMs) are rare pathologies compared with radiation-induced cerebral CMs. We present a case of a radiation-induced spinal cord CM developed 31 years after radiation therapy for medulloblastoma., Case Description: A 37-year-old man developed a symptomatic spinal hemorrhagic lesion 31 years after radiation therapy for medulloblastoma. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the C7 area. Surgery was performed leading to an unclear diagnosis. Two years later, the patient had a relapse and underwent a second operation, allowing a definitive diagnosis of radiation-induced spinal cord CM. This is believed to be the second case of de novo intramedullary CM formation following spinal radiation therapy for medulloblastoma., Conclusions: Radiation-induced spinal cord CMs should be recognized as a possible late adverse effect in patients treated with radiation therapy for medulloblastoma., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Hemangioblastomas are uncommon tumors of the central nervous system that can be seen in Von Hippel-Lindau (VHL) disease. Despite their benign histology, hemangioblastomas can cause substantial morbidity due to involvement of critical structures. In order to better understand the clinical behavior of spinal cord hemangioblastomas, we have analyzed the clinical, pathologic, radiologic characteristics and management of sporadic and VHL-associated cases at our institution., Methods: We performed a database search to identify all spinal hemangioblastomas at our institution between 1997 and 2016. Tumor characteristics were analyzed for sporadic and VHL-associated tumors separately in order to understand the differences in groups., Results: We included 20 patients with VHL-associated spinal hemangioblastomas, and 22 patients with sporadic spinal hemangioblastomas. VHL-associated patients were significantly younger at time of presentation compared to sporadic patients (p < 0.0025). Thirty-two patients (76.2%) presented with focal weakness, 34 (81.0%) with sensory loss, and 22 (52.4%) with pain. VHL patients were more likely to present with multiple symptoms (p < 0.001). Median follow-up time was 20.9 months, during which 17 tumors recurred. The median recurrence free interval was 44 months. There were no differences in gross total resection rates between sporadic and VHL-associated cases (p = 0.197). VHL-associated cases had a higher rate of repeat surgery for recurrence (14 patients-73.6%) compared to sporadic cases (3 patients-13.6%; p < 0.001)., Conclusion: VHL-associated spinal hemangioblastomas differ from sporadic tumors in terms of age, presenting symptoms, multifocality, and rate of recurrence. Recurrences seem to be unrelated to the extent of resection, indicating the need for life-long follow up for VHL patients.

Compared to cerebral radiation-induced cavernous hemangiomas (RICHs), little is known about intraspinal RICHs. A 13-year-old male suddenly developed symptomatic spinal hemorrhage eight years after hematopoietic stem cell transplantation using a total body irradiation (TBI) based myeloablative regimen. A solitary small hemangioma was detected on follow-up T2 star weighted magnetic resonance imaging of the spine. His neurological symptoms gradually improved with supportive treatment and rehabilitation, although he experienced rebleeding 2 years later. Intraspinal RICH is very rare but should be recognized as a possible late adverse effect in pediatric patients who received TBI., (© 2018 Wiley Periodicals, Inc.)

Objective: Hemangioblastomas (HBs) are benign vascular tumors of the central nervous system and histologically contain abundant microvessels. Therefore, they clinically exhibit vascular malformation-like characteristics. It has been described that endothelial-to-mesenchymal transition (EndMT) contributes to the pathogenesis of cerebral cavernous malformations. However, it remains unknown whether EndMT contributes to the pathogenesis of central nervous system HBs. The aim of our study was to investigate whether EndMT occurs in central nervous system HBs., Methods: Ten central nervous system HBs were immunohistochemically investigated., Results: Cluster of differentiation (CD) 31 (an endothelial marker) and EndMT markers, such as α-smooth muscle actin (a mesenchymal marker) and CD44 (a mesenchymal stem cell marker), were expressed in the endothelial layer of microvessels in all cases. These findings suggest that endothelial cells (ECs) of microvessels in central nervous system HBs have acquired mesenchymal and stem cell-like characteristics and undergone EndMT. In all cases, both ephrin-B2 and EphB4, which are not detected in adult normal brain vessels, were expressed in the endothelial layer of microvessels. These data suggest that ECs of microvessels in central nervous system HBs are immature or malformed cells and have both arterial and venous characteristics., Conclusions: To our knowledge, this is the first report showing the possibility that EndMT contributes to the pathogenesis of central nervous system HBs. It is likely that ECs of microvessels in central nervous system HBs are immature or malformed cells and have both arterial and venous characteristics. EndMT is expected to be a new therapeutic target in central nervous system HBs., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pediatric hemangioblastomas are rare, and the clinical features, timing of surgical intervention, optimal treatment, and clinical outcomes are still unclear., Methods: We performed a retrospective study of all patients with CNS hemangioblastomas who were treated at West China Hospital from January 2003 to March 2015. Patients under the age of 16 years were included in the study. The medical records of these patients were reviewed and statistically analyzed., Results: Twenty-five children (15 females and ten males, [mean age 12.6 ± 4.7 years, range 1-16 years]) presented with hemangioblastomas. Tumors were detected in the cerebellum, brainstem, and spinal cord in 40, 28, and 32% of patients, respectively. Sixteen children (64%) had VHL syndrome. The most frequent symptoms were those related to increased intracranial pressure. The mean duration of symptoms was 1.5 ± 2.1 months. Preoperative hydrocephalus was noted in 11 children (44%). Gross total resection was achieved in all children. Clinical symptoms improved in 19 children (76%), unchanged in four children (16%), and aggravated in two children (8%), respectively. The mean follow-up was 44.5 ± 32.3 months. Five patients (20%) experienced disease progression. Using univariate analysis, both tumor-associated cysts (P = 0.027) and VHL disease (P = 0.032) were significantly related to postoperative outcomes., Conclusions: Pediatric hemangioblastomas have many different clinical features compared with adult cases. A high degree of suspicion for VHL disease should be raised in pediatric hemangioblastomas. Despite many challenges involved, surgical outcomes for pediatric hemangioblastomas are favorable. Lifelong follow-up is mandatory to detect the disease progression.

Opinion Statement: Malignant epidural spinal cord compression (MESCC) is an oncologic emergency with the potential for devastating consequences for patients if not promptly diagnosed and treated. MESCC is diagnosed by imaging. MRI is by far the most sensitive test, preferably with gadolinium. Once the diagnosis of MESCC is suspected, patients with neurologic deficits should receive prompt administration of dexamethasone with a 10-mg IV loading dose followed by 4 mg every 6 h. Quick taper is recommended once the definitive treatment is established. Consultation with medical oncology, radiation oncology, and neurosurgery is imperative in order to facilitate a multidisciplinary approach. Although spine surgery is the most effective method for relief of cord compression and is necessary if there is spinal instability, surgery is only used in selected patients because most patients have a poor overall condition and short life expectancy. Radiation therapy, therefore, is the most commonly used therapy for patients with MESCC after surgical decompression or in patients who are not surgical candidates. Conventional fractionated radiation alone can achieve modest neurologic outcomes in selected radiosensitive tumors. Radiosurgery techniques which deliver intense focal irradiation to a delimited area with imaging guidance and contoured radiation delivery to the shape of the tumor have recently emerged as increasing effective treatments in MESCC, especially in radioresistant tumors. Stereotactic radiosurgery and different radiation technologies have been studied in recent clinical trials.

Background: Spinal epidermoid cysts are benign tumors, which are rarely seen as an intradural extramedullary spinal cord tumor in the conus medullaris region. Acquired spinal epidermoid cysts are mostly caused by iatrogenic procedures, such as lumbar puncture, and the majority of acquired spinal epidermoid cysts have been reported below the L1 level, because lumbar puncture is usually performed around the iliac crest. Here, we report an extremely rare case of an epidermoid cyst that occurred as an intradural and extramedullary spinal cord tumor attached to the conus medullaris after repetitive epidural anesthesia., Case Presentation: A 67-year-old female presented with a low back pain and left sciatica. Although the patient had experienced occasional mild low back pain for several years, her low back pain markedly worsened 2 months before her visit, as well as newly developed left sciatica resulting in intermittent claudication. She had a history of several abdominal surgeries. All abdominal procedures were performed under general anesthesia with epidural anesthesia in her thoracolumbar spine. Magnetic resonance imaging of her lumbar spine demonstrated an intradural extramedullary spinal cord tumor at the T12-L1 level. Because her symptoms deteriorated, the tumor excision was performed using microscopy. Histological examination of the specimens demonstrated that the cyst walls lined with stratified squamous keratinizing epithelium surrounded by the outer layer of collagenous tissue with the absence of skin adnexa. A diagnosis of epidermoid cysts was confirmed. Her MRI showed complete resection of the tumor, and there was no recurrence at 2-year follow-up., Conclusions: In this case report, epidermoid cells might be contaminated into the spinal canal during repetitive epidural anesthesia. The patient was successfully treated by complete resection, and there was no recurrence at 2-year follow-up with a good clinical outcome. However, long-term follow-up is required for a potential risk of tumor recurrence.

Central neurocytomas comprise nearly half of adult intraventricular neoplasms. The median age of onset is 34 years. It is typically a low-grade neoplasm (World Health Organization grade II), although some cases of malignant neurocytomas have been described. We present a rare case of an atypical central neurocytoma with craniospinal dissemination, including both imaging and pathologic findings., (Published by Elsevier Inc.)

Cavernous angiomas of the spinal cord exhibit imaging characteristics that may overlap with those of hemorrhagic ependymoma. In the present study, we aimed to identify specific magnetic resonance imaging (MRI) findings that could be used to differentiate cavernous angioma from hemorrhagic ependymoma, and to evaluate serial MRI changes in cases of cavernous angioma. We retrospectively evaluated MR images of spinal cord tumors collected at our hospital from 2007 to 2015. From this cohort of images, 11 pathologically confirmed cavernous angiomas and 14 pathologically confirmed hemorrhagic ependymomas were compared with respect to the size of the tumor, longitudinal location, axial location, enhancement pattern, syrinx, edema, tumor margin, signal intensity of T2WI, signal intensity of T1WI, and longitudinal spreading of the hemorrhage. Serial MR images of seven spinal cavernous angiomas were reviewed. Small size, eccentric axial location, minimal enhancement, and absence of edema were more frequently observed on images of cavernous angioma compared to those of hemorrhagic ependymoma (p < 0.01). Serial MRI changes in cases of cavernous angioma included increased longitudinal spreading of the hemorrhage (6/7, 86 %) and emergence of high signal intensity on T1WI (1/7, 14 %). Small size, eccentric axial location, minimal enhancement, and absence of edema are significant MRI findings that may be used to distinguish Type I and Type II spinal cavernous angiomas from hemorrhagic ependymomas. Furthermore, longitudinal spreading of the hemorrhage may be observed on follow-up MRIs of cavernous angiomas.

Background/aim: Adrenocortical cancer is a rare aggressive type of cancer. The prognosis is poor, particularly for metastatic disease. This study focused on metastatic spinal cord compression (MSCC) from adrenocortical carcinoma., Patients and Methods: Data of three patients who received palliative irradiation of MSCC from adrenocortical carcinoma were retrospectively analyzed for motor function, ambulatory status and survival., Results: One patient died before completion of radiotherapy. The other two patients died two weeks and four weeks, respectively, following irradiation. In these patients, pre-radiotherapy pain scores were 9 and 10 points. In both patients, partial pain relief was achieved (scores of 5 and 4 points). All three patients were non-ambulatory before irradiation. In assessable patients, motor function remained unchanged following irradiation., Conclusion: Palliative irradiation resulted in considerable pain relief, whereas motor function did not improve. Considering the extremely poor survival, supportive care alone may be considered if pain relief is achieved without irradiation., (Copyright © 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.)

Background: Kabuki syndrome is a rare disorder characterized by the association of mental retardation and postnatal growth deficiency with distinctive facial appearance, skeletal anomalies, cardiac and renal malformation. Two causative genes have been identified in patients with Kabuki syndrome. Mutation of KMT2D (MLL2) was identified in 55-80% of patients, while 9-14% of KMT2D negative patients have mutation in KDM6A gene. So far, few tumors have been reported in patients with Kabuki syndrome. We describe the first case of a patient with spinal ependymoma and Kabuki syndrome., Case Presentation: A 23 years old girl followed at our Center for KMT2D mutated Kabuki syndrome since she was 4 years old presented with acute lumbar pain and intermittent tactile hyposthenia of the feet. Spine magnetic resonance revealed a lumbar endocanalar mass. She underwent surgical resection of the lesion and histologic examination showed a tanycytic ependymoma (WHO grade II)., Conclusion: Kabuki syndrome is not considered a cancer predisposition syndrome. Nonetheless, a number of tumors have been reported in patients with Kabuki syndrome. Spinal ependymoma is a rare disease in the pediatric and young adult population. Whereas NF2 mutations are frequently associated to ependymoma such an association has never been described in Kabuki syndrome. To our knowledge this is the first case of ependymoma in a KMT2D mutated Kabuki syndrome patient. Despite KMT2D role in cancer has previously been described, no genetic data are available for previously reported Kabuki syndrome patients with tumors. Nonetheless, the association of two rare diseases raises the suspicion for a common determinant.

Peripheral nerve sheath tumours are a well-recognised complication of radiotherapy. Single tumours presenting up to 10 years after initial treatment have been previously described. We report an individual with two anatomically separate nerve sheath tumours developing at different time intervals following initial treatment, after 38 and 42 years, respectively. This case illustrates the importance of maintaining vigilance for the development of complications even at an advanced stage following index radiotherapy treatment., (2015 BMJ Publishing Group Ltd.)

A 4-year-old girl with a history of thoracic meningocele repair at the age of 3 months presented with progressive myelopathy. An intramedullary thoracic epidermoid was identified on MRI. The patient underwent excision of the epidermoid and subsequently returned to neurological baseline. This case illustrates the potential for delayed development of intraspinal epidermoid after initial repair of a simple meningocele.

Study Design: This is a case report., Objectives: Spinal cord atrophy presenting with late progressive myelopathy after many years of clinical stability is a rare and unexplained phenomenon after cervical spine surgery. The authors report and discuss the etiologies and outcomes of late postoperative myelomalacic myelopathy., Setting: This study was conducted in the Department of Neurosurgery, Centro Hospitalar S. João, Porto, Portugal., Methods: We report on two patients with insidious chronic progressive myelopathy that developed more than 10 years after complete removal of a cervical intramedullary ependymoma and after transoral odontoidectomy and occipitocervical fusion for craniocervical junction malformation. Differential diagnoses were formulated and review of the literature was performed., Results: In both patients, after several years of clinical stability, insidious onset of debilitating myelopathy and dependency ensued. The clinical history, serology, imaging and neurophysiological investigation excluded several putative etiologies: arachnoid adhesions, tumor recurrence, late vertebral instability, trauma, syringomyelia, radiotherapy, and demyelinating or infectious causes., Conclusion: Late neurological deterioration after cervical spine surgery is usually related to disease progression or surgery-related complications. Nevertheless, in some patients late myelopathy can ensue even in the absence of identified causes.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline., (© 2015 S. Karger AG, Basel.)

We report a 44-year-old with progressive quadriparesis due to a dumbbell malignant peripheral nerve sheath tumour (MPNST) of the second cervical nerve, 17 years after whole brain radiotherapy for a pineal germinoma. To our knowledge this is the first case of radiation induced high cervical MPNST arising from a benign neurofibroma., (Crown Copyright © 2014. Published by Elsevier Ltd. All rights reserved.)

Study Design: Marjolin's ulcer is a squamous cell carcinoma that develops in posttraumatic scars and chronic wounds. Suspicion of such lesions should be raised in chronic wounds demonstrating characteristic changes. We have reported the peculiar phenomenon of malignant transformation of chronic pressure sores that occurred in a paraplegic patient., Objectives: The aim of this study was to cover the extensive defects by a last resort reconstructive option., Setting: Department of Plastic and Reconstructive Surgery, Università Politecnica delle Marche, Ancona, Italy., Methods and Results: A 40-year-old paraplegic man, with multiple hemangioblastomas of the brain and spinal cord due to Von Hippel Lindau syndrome developed pressure ulcers with unstable healing over the sacral, trochanteric, bilateral, and ischiatic areas after 15 years from neurosurgery. The biopsy result showed an invasive squamous carcinoma. Carcinomas in pressure sores are highly aggressive, and they need to be treated more radically. In our case we opted for a demolitive surgical treatment including musculocutaneous rotational flap harvested from total left thigh to cover the extensive defects. The limb was previously disarticulated., Conclusion: In Marjolin's ulcer, multiple biopsies are the first-line modality for the early diagnosis as they are a safe method with high rate of accuracy. First-line treatment is surgery consisting of radical excision with lymph node dissection, if they are involved. Adjuvant radiation therapy may be used in selected patients. Management of massive pelvic defects can be a challenging problem. The pedicled lower limb flap offers a technique that can be considered as a last resort procedure for extensive defects where other options are insufficient or not available anymore. In our case the patient is disease-free after 2 years of follow-up.

Introduction: Obstructive sleep apnea (OSA) was associated with increased incidence of all cancers. We aimed to determine the risk for primary central nervous system (CNS) cancers in patients with sleep apnea syndrome., Methods: A total of 23,055 incident cases of newly diagnosed sleep apnea syndrome (sleep apnea group) were identified between 2000 and 2003 in the medical claims database of Taiwan's National Health Institute (NHI) program and were matched by age and gender to patients without OSA (comparison group) in the same period. The occurrence of primary malignant CNS cancers was measured 2 years after the index date over a 10-year period., Results: The incidence density of primary CNS cancers (per 10,000 individual-years) was 2.14 and 1.28, respectively, for the OSA and comparison groups. The overall risk for developing primary CNS cancers was significantly higher in the OSA group (adjusted hazard ratio [HR], 1.54; P=0.046) after adjusting for age, gender, and obesity, among other variables. Subgroup analysis revealed a significantly higher risk for primary brain cancers but not primary spinal cord cancers in the OSA subgroup (adjusted HR, 1.71; P=0.027). The analysis also revealed a significantly higher risk for primary CNS cancers in the insomnia with OSA subgroup (adjusted HR, 2.20; P=0.001) and in the OSA without surgical treatment subgroup (adjusted HR, 1.831; P=0.003)., Conclusions: OSA, especially with insomnia, may increase the risk for primary CNS cancer development, though surgical treatment may reduce this risk in participants with OSA., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Primary intramedullary spinal cord tumors are a rare entity, comprising 4–10 %of all spinal cord tumors. The current report presents data on intramedullary spinal cord anaplastic astrocytomas and glioblastomas in adults using the national surveillance, epidemiology, and end results database (1973–2008), and evaluates the impact of demographic and treatment factors on survival. Eighty nine adults were evaluated (mean age of 43 years); 49 % of patients had anaplastic astrocytoma and 51 % of patients had glioblastoma.88 % of patients had surgical intervention and 85 % of patients had radiotherapy. In univariate analysis, male gender (HR = 0.50, CI: 0.29–0.86, P = 0.01), surgical treatment (HR = 0.37, CI: 0.15–0.93, P = 0.03), and tumor histology (HR = 1.83, CI: 1.06–3.18, P = 0.03) were significant predictors of survival. Results remained significant or marginally significant after multivariate adjustment analyses. Adjuvant radiotherapy and age at diagnosis did not have a significant influence on survival. Future prospective studies from collaborative institutions combining richer detail in perioperative treatment, radiotherapy dosing, chemotherapy treatment, neurologic examinations, functional outcomes, and quality of life measures would contribute to more concrete, evidence-based treatment protocols for adult patients with primary malignant spinal cord astrocytomas.

Background: Intramedullary hemangioblastomas are rare benign tumors, occurring sporadically or in von Hippel- Lindau disease., Methods: We describe our local surgical experience with intramedullary hemangioblastomas. Clinical, imaging and surgical data from five consecutive hemangioblastoma cases identified from a series of 59 patients with intramedullary tumors treated between 2003-2009 are reviewed., Results: The mean age of the patients was 39.6 years (range 21- 56). All of them were symptomatic and two patients had von Hippel-Lindau disease with associated posterior fossa hemangioblastomas. All tumors were preoperatively diagnosed as hemangioblastomas based on magnetic resonance findings. All patients underwent surgery with complete removal of the tumor in 4 cases and a partial removal in a case with extension towards the anterior part of the cord. Good neurological outcome was noted in four cases while in the fifth, complicated with a significant intraoperative hemorrhage, a fully reversible aggravation of neurological status occurred., Conclusions: Spinal cord hemangioblastomas are surgically curable tumors. Microsurgical complete resection is the standard of care and can be performed with good neurological outcome in most of the cases. Ventral tumor location and important intraoperative bleeding are associated with less optimal outcome., (Celsius.)

Glioblastoma multiforme (GBM) is the most common malignant primary brain tumor in adults. Compared with other malignancies, remote metastases in GBM are rare. However, multicentric spreading within the central nervous system is common and also metastases to the spinal cord have been reported. Some of these drop metastases may also lead to malignant spinal cord compression (MSCC). We retrospectively identified nine patients from 2001 to 2010 and performed data analysis according to a standardized clinical protocol. We also provide a review of the literature on this rare condition. MSCC from cerebral GBM is rare and is found in approximately 1 % of GBM patients. Median age of 54 years in this case series is comparable with that of GBM patients without MSCC. Treatment regimens for cerebral GBM and overall survival was similar to those for patients without MSCC. Spinal metastasis seems to occur in the advanced state of the disease, and the outcome subsequently is extremely poor. All patients presented with multicentric radiological features of GBM on cerebral MRI when MSCC was diagnosed. Subependymal enhancement is another common radiological finding in GBM patients with spinal drop metastases. Steroids and focal radiotherapy were used to treat all patients, with little clinical benefit. This study is the largest case series of MSCC from cerebral GBM. Multicentric cerebral distribution and subependymal enhancement of GBM are observed on cerebral MRI at the time of MSCC. On the basis of our results, no specific treatment recommendations for MSCC in GBM patients can be given. However, accurate diagnosis of MSCC in GBM patients with spinal signs and symptoms can lead to adequate management of symptoms and improvement of quality of life in terms of best palliative care.

We describe the clinical features of limbic encephalitis that developed after palliative spinal cordotomy in 2 patients with malignant thoracic astrocytoma. Both patients showed short-term memory loss, hallucinations of smells and psychiatric symptoms. Brain MRI on T2-weighted and fluid-attenuated inversion recovery sequences revealed high intensity lesions in bilateral temporal lobe areas. We considered that both patients had paraneoplastic limbic encephalitis associated with astrocytoma because of various clinical and radiological features. But the possibility of intracranial dissemination of astrocytoma could not be fully excluded. The differential diagnosis between intracranial dissemination of spinal cord astrocytoma and paraneoplastic limbic encephalitis may be sometimes difficult.

A 54-year-old man presented with a very rare case of radiation-induced intramedullary spinal cord anaplastic astrocytoma, which developed 37 years after radiotherapy for testicular seminoma. The patient presented with weakness and numbness of the left lower extremity that had gradually aggravated for 3 months. Magnetic resonance imaging demonstrated an intramedullary mass lesion with syringomyelia at the T9 to T12 levels. Subtotal removal of the tumor was performed using standard microsurgical technique. Histological examination revealed anaplastic astrocytoma. Although radiotherapy was seriously considered, chemotherapy was employed as adjuvant therapy considering the previous treatment. Although his neurological status improved transiently after surgery, relentless neurological decline occurred and resulted in death 9 months following surgery. Considering that subtotal removal of the tumor and chemotherapy had little influence on the quality of life and the length of survival in our case, cordectomy may be the optimum treatment for patients with radiation-induced spinal intramedullary malignant astrocytoma.

Patients with von Hippel-Lindau (VHL) syndrome with diffuse CNS hemangioblastomas have morbidity related to their disease and require a lifetime of surgical resections. Ninety-seven percent of tumors progress, and 5-year surgery rates are 20%-60%. Stereotactic radiosurgery and fractionated radiotherapy have had limited success. For the first time, we have used infratentorial craniospinal radiation therapy (ICSRT) for VHL patients with CNS hemangioblastomas. Consecutive VHL patients treated at the National Institutes of Health with radiographic evidence of hemangioblastomas were included if they received ICSRT. Patients underwent neurologic examinations and imaging at 3- to 12-month intervals. Seven patients with 84 hemangioblastomas met eligibility criteria. ICSRT was commonly administered to 43.2 Gy in 24 fractions. Mean pre-ICSRT tumor volume was 5.48 cm(3). At a mean follow-up of 73.8 months, mean post-ICSRT tumor volume was 6.87 cm(3), and 91 tumors were identified. Complete radiographic resolution was achieved in 17.9% of lesions. Although many patients were no longer optimal surgical candidates, only 4 surgeries were needed for symptomatic lesions after ICSRT, compared with 33 prior. Acute toxicity was mild and no patient developed grade ≥1 late spinal cord toxicity according to the criteria of the Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer, despite the high dose that the entire spinal cord received. Clinical and radiographic stability or resolution was demonstrated in the majority of tumors. Tumor growth rate in this study was less than reported in natural history studies, and the rate of surgical intervention was reduced. ICSRT was well tolerated, can decrease hemangioblastoma growth rate, and is a potential therapeutic option for VHL patients that warrants further investigation.

Background: Central nervous system (CNS) hemangioblastomas are a benign condition, which can be permanently cured by complete surgical removal. However, the vascular nature of these lesions and difficulties in localizing the tumors account for operative morbidity and recurrence. Power Doppler flow sonography has been proven useful during surgical removal of other vascular lesions., Objective: To evaluate the usefulness of Power Doppler flow sonography for hemangioblastoma., Methods: We used the SonoWand Invite (Sonowand AS, Trondheim, Norway) intraoperative navigation system in a consecutive series of hemangioblastomas operated on at our institution. Patients with von Hippel-Lindau (VHL) disease as well as sporadic hemangioblastomas were included., Results: The system was used on n = 64 consecutive hemangioblastomas operated on at our institution from 2007 to 2009. The tumors were localized in the cerebellum (n = 26), spinal cord (n = 27), brainstem (n = 10), and supratentorial (n = 1). In VHL disease was diagnosed 53 patients, and germline mutations of the VHL tumor suppressor gene were identified in 98%. Average tumor size was 1782 mm and 45% of the tumors were cystic. Forty-two of 64 tumors could be localized by grayscale sonography. All tumors were visible on power Doppler flow sonography. However, in 40 cases, only the pathological vessels and not the solid tumor itself enhanced on power Doppler. Postoperative MRI follow-up revealed remnant/recurrent tumors in 2 cases., Conclusion: Power Doppler flow sonography is a sensitive intraoperative tool to guide the surgical approach and resection and provides reliable resection control in surgery of CNS hemangioblastoma.

Object: The incidence of ependymoma in patients with neurofibromatosis-2 (NF-2) is low and information regarding treatment and prognosis is lacking. We present two cases of cervicomedullary tumors in patients with NF-2 from our institution, and we provide a review of the literature in order to summarize the known clinical information about this rare occurrence., Patients and Methods: Patient #1 had histological confirmation of ependymoma and was treated with subtotal resection followed by observation and has had no evidence of progression for 11 months. Patient #2 has been observed for 4 1/2 years without treatment for a cervicomedullary tumor, which appears to be an ependymoma by imaging. Although it has increased in size very slowly, there have been no clinical symptoms. Among the additional 21 cases of NF-2 and ependymoma from the literature, the most common location is the cervical spine (70%), and the median age at diagnosis is 15 years. Surgical resection was performed in 85% of the cases and subtotal resection in 64% of cases. Fifteen patients (75%) were reported alive at the time of the published reports, with survival ranging from 0.1 to 10 years, and the 8-year survival estimated as 51%. Survival was related to the location of the tumor., Conclusions: We conclude from our two cases and review of the existing literature that NF-2 associated spinal ependymomas have an indolent course and typically can be observed or treated by surgical excision alone.

Intravascular lymphoma is a rare disorder that commonly involves the central nervous system. Neurologic involvement may be the presenting and only manifestation. Identifying intravascular lymphoma as the cause of neurologic disease is diagnostically challenging. We report an elderly woman presenting with subacute onset paraparesis due to spinal cord involvement by an intravascular lymphoma. Progressive worsening was associated with extension of a longitudinally-extensive thoracic intramedullary spinal cord lesion. Extensive investigations failed to provide a diagnosis in life and repeated empiric therapeutic trials were unsuccessful. Diagnostic confirmation was postmortem. A longitudinally-extensive spinal cord lesion has a broad differential diagnosis. Intravascular lymphoma should be considered particularly in older individuals. The presence of coexisting hematologic abnormalities should prompt consideration of a bone marrow biopsy. Early diagnosis may direct therapy and lead to a more favorable prognosis., (Copyright © 2010 Elsevier B.V. All rights reserved.)

Von Hippel-Lindau disease (VHL) comprises a series of complicated clinical manifestations. We hereby report one unique case of VHL with a natural history that mimics acute myelitis. MRI and biopsy in this patient showed multiple solid hemangioblastomas of the central nervous system and kidney. This study further confirmed that VHL is of highly clinical, imaging, and pathological heterogeneity. Diagnosis for VHL should be based on combination of clinical, radiological, pathological, and genetic data.

Background Context: Radiation-induced meningiomas (RIMs) of the brain are the most common RIMs. However, there has been no report in the literature of an RIM of the spine. Conus medullaris is a favorite site for ependymomas, whereas it is an extremely rare location for meningiomas. Dumbbell-shaped configuration is typical for nerve sheath tumors; however, it is very rarely seen in meningiomas., Purpose: To describe imaging findings of a possibly RIM at the level of the conus medullaris mimicking a neurofibroma., Study Design: A 60-year-old male with 6-year history of irradiated urinary bladder cancer presented with paraparesis., Methods: Radiography, computed tomography, and magnetic resonance imaging of the thoracolumbar spine were undertaken., Results: Radiography showed widening of intervertebral foramina at T12-L1 level. Computed tomography and magnetic resonance imaging disclosed a lobulated intradural mass at the level of the conus medullaris causing widening and scalloping of adjacent bony structures, with dumbbell-shaped configuration. Surgical biopsy of the mass was consistent with fibroblastic meningioma., Conclusions: The reported tumor might have developed secondary to irradiation for urinary bladder cancer because meningiomas are the most common radiation-induced central nervous system tumors. Although location at the level of the conus medullaris is atypical for meningioma, and dumbbell-shaped configuration is rather characteristic for neurogenic tumors, they should not exclude a diagnosis of meningioma., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: Neurofibromatosis type 2 (NF2) is an inherited, rare autosomal dominant syndrome characterised by the development of multiple benign cranial and spinal tumours, peripheral neuropathy, ophthalmological and cutaneous lesions. Herein, we report one case of NF2 treated with multivariate chemotherapy., Material and Methods: A 13-year-old female presented with multiple cranio-spinal tumours in MRI. First symptoms were progressive changes in vision, left-sided paresis, unilateral sensorineural hearing loss, and left hypoglossal nerve paresis. The patient underwent palliative, partial surgical resection of the tumour which was located in a posterior fossa. Histopathological examination showed a psammomatous meningioma located near the great foramen and schwannomas of VIII nerve in the cerebello-pontine angle. Clinical and radiological examination revealed a rapid progression of the disease. As such, multivariate chemotherapy was used. The patient died 4 years after diagnosis., Conclusion: NF2 patients with multiple tumours at diagnosis may not be treatable with surgery alone and, as a result, presentation with such a disease in childhood results in poor prognosis. The unification of management strategies in NF2 patients is highly desirable.

We report a rare case of a woman with bilateral papillary cystadenomata of the broad ligament with von Hippel-Lindau disease (VHL) (other manifestations: capillary hemangioblastomas of the spinal cord). Patient surveillance is important, because in the course of VHL-associated tumors malignant lesions may arise that are relevant for the prognosis.

Swelling of the spinal cord and/or enhancement after intravenous gadolinium administration are not always specific features of intramedullary tumour. These may also be seen in association with several diseases of inflammatory, infectious, granulomatous or vascular origin. A tumour is characterized by its sagittal location, axial topography: central, lateral or exophytic, its size and size of the spinal canal, macroscopic components: calcium, fat, methemoglobin, melanin, hemosiderin, vascular pedicle, cystic component, enhancement after intravenous gadolinium administration, effect on the spinal cord tracts and edema. Characteristics: astrocytoma is lateral and infiltrative, ependymoma is central with white matter tract displacement and hemosiderin cap, hemangioblastoma is postero-lateral and shows enhancement with a vascular pedicle, metastases are very edematous or leptomeningeal in location.

Objective: Late neurologic deterioration after brachial plexus injury (BPI) is uncommon and may be caused by multiple etiologies. An unusual, previously unreported, case of late neurologic deterioration after BPI is presented., Methods: A pediatric patient previously treated for a traumatic preganglionic BPI and presenting with neurologic deterioration 5 years after his injury is reviewed., Results: Magnetic resonance imaging and computed tomography myelogram revealed spinal cord herniation at the same level of the previous nerve root avulsions. Surgical open biopsy demonstrated a spinal cord anaplastic astrocytoma. Despite craniospinal radiotherapy and different regimens of chemotherapy, he died 4 years later from leptomeningeal spread., Conclusion: This case illustrates a previously unreported cause of late neurologic deterioration following BPI. The probable coincidental versus possible causal interrelationship of these two processes (BPI and spinal cord tumor) is discussed, but no conclusions can be reached., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Tanycytic ependymoma is a rare subtype of ependymoma. Reports of this tumor in neurofibromatosis type 2 (NF-2) are rare. A 16-year-old girl presented with gait disturbance and a palpable neck mass, which had been present for 2 years. MRI revealed an intramedullary lesion within the upper cervical spinal cord, which was removed surgically. Pathological investigation revealed an uncommon form of tanycytic ependymoma associated with NF-2. This rare morphology of tanycytic ependymoma could be misinterpreted as pilocytic astrocytoma or other tumor types that exhibit elongated cells. Increased awareness of this transitional form of intramedullary ependymoma among neurosurgeons and pathologists might avoid incorrect surgical approaches and postoperative treatments.

Object: Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas., Methods: Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed., Results: One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients)., Conclusions: Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.

Summary: In patients with Down syndrome, cancers like leukemia and testicular tumors are frequent, but association with central nervous system tumors is rare. Only 1 case of ependymoma has been observed as an incidental autopsy finding in a 19-week-old female fetus. We herein report the second case of ependymoma and the fifth case of spinal tumor occurring in association with Down syndrome. We have also attempted to elucidate the various mechanisms of tumorigenesis implicated in this multiple malformation syndrome. A 13-year-old girl with Down syndrome presented with progressively increasing paraparesis and neurogenic bladder. Magnetic resonance imaging of dorsolumbar spine revealed an intramedullary mass (L1 to L5 level). The patient underwent near total excision of tumor with postoperative histopathology showing myxopapillary ependymoma. Karyotyping showed classic Down syndrome with trisomy 21. Postoperative irradiation (45 Gy in 25 fractions over 5 wk followed by boost up to 55 Gy) was subsequently delivered. One year after the completion of the tumor-directed therapy, the patient is in radiologic complete remission, with improved power in both lower limbs. Association of ependymoma with Down syndrome is a rarity, which at best, can be explained as a chance phenomenon.