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In the most recent fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System, astroblastoma has been defined by molecular rearrangements involving the MN1 gene, with common partners being BEND2 or CXXC5 . Accordingly, this tumor entity is now known as "astroblastoma, MN1 -altered." However, gliomas with EWSR1::BEND2 fusions, devoid of MN1 fusion alterations, have recently been shown to exhibit astroblastoma-like histomorphologic features and reside in a distinct epigenetic subgroup based on DNA methylation studies similar to high-grade neuroepithelial tumor with MN1 alteration, which includes astroblastoma, MN1 altered tumors. This new epigenetically distinct subtype of astroblastoma containing EWSR1::BEND2 fusions lacks the required MN1 alteration and, thus, does not satisfy the current molecular classification of these lesions. Here, we describe a case of glioma with histologic features and DNA methylation profiling consistent with astroblastoma with a novel YAP1: : BEND2 fusion. This case and others further expand the molecular findings observable in astroblastoma-like tumors outside the constraints of MN1 alteration. Such cases of astroblastoma with EWSR1::BEND2 and YAP1::BEND2 fusions challenge the current molecular classification of astroblastoma based solely on an MN1 alteration.
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Proteínas Adaptadoras de Transdução de Sinal , Neoplasias Neuroepiteliomatosas , Proteínas de Fusão Oncogênica , Fatores de Transcrição , Proteínas de Sinalização YAP , Humanos , Neoplasias Neuroepiteliomatosas/genética , Neoplasias Neuroepiteliomatosas/patologia , Fatores de Transcrição/genética , Proteínas Adaptadoras de Transdução de Sinal/genética , Proteínas de Fusão Oncogênica/genética , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Masculino , Metilação de DNA , Fosfoproteínas/genética , FemininoRESUMO
Background: Choroid plexus tumors (CPTs) are rare neoplasms found in the central nervous system, comprising 1% of all brain tumors. These tumors include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Although gross total resection for choroid plexus papillomas (CPPs) is associated with long-term survival, there is a scarcity of prospective data concerning the role and sequence of neoadjuvant therapy in treating aCPP and CPC. Methods: From the years 2000 to 2019, 679 patients with CPT were identified from the Surveillance, Epidemiology, and End Result (SEER) database. Among these patients, 456 patients had CPP, 75 patients had aCPP, and 142 patients had CPC. Univariate and multivariable Cox proportional hazard models were run to identify variables that had a significant impact on the primary endpoint of overall survival (OS). A predictive nomogram was built for patients with CPC to predict 5-year and 10-year survival probability. Results: Histology was a significant predictor of OS, with 5-year OS rates of 90, 79, and 61% for CPP, aCPP, and CPC, respectively. Older age and African American race were prognostic for worse OS for patients with CPP. Older age was also associated with reduced OS for patients with aCPP. American Indian/Alaskan Native race was linked to poorer OS for patients with CPC. Overall, treatment with gross total resection or subtotal resection had no difference in OS in patients with CPP or aCPP. Meanwhile, in patients with CPC, gross total resection (GTR) was associated with significantly better OS than subtotal resection (STR) only. However, there is no difference in OS between patients that receive GTR and patients that receive STR with adjuvant therapy. The nomogram for CPC considers types of treatments received. It demonstrates acceptable accuracy in estimating survival probability at 5-year and 10-year intervals, with a C-index of 0.608 (95% CI of 0.446 to 0.77). Conclusions: This is the largest study on CPT to date and highlights the optimal treatment strategies for these rare tumors. Overall, there is no difference in OS with GTR vs. STR in CPP or aCPP. Furthermore, OS is equivalent for CPC with GTR and STR plus adjuvant therapy.
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INTRODUCTION: Atypical teratoid rhabdoid tumor (ATRT) is among the most aggressive central nervous system malignancies. Although rare, this tumor typically afflicts young children and results in mortality within months. Here, we aim to determine key clinical features and treatment options that impact the survival of patients with ATRT. METHODS: From the year 2000 to 2019, 363 patients with ATRT were identified from the Surveillance, Epidemiology, and End Results database. Univariate analysis was used to identify variables that had a significant impact on the primary endpoint of overall survival (OS). Multivariable analysis was then used to identify independent predictors of survival. RESULTS: The median OS of the entire cohort was 13 months. Univariate analysis identified ages between 1 and 3 years, ages between 4 and 17 years, years of diagnosis between 2010 and 2019, and the receipt of treatment to have a significant impact on survival. In multivariable analysis, ages between 1 and 3 years and receipt of treatment were the only significant independent predictors of survival. The median OS was significantly greater in patients who received surgical treatment, chemotherapy, or radiation when compared to those who did not receive any treatment. In general, the receipt of any combination of therapies improved the median OS significantly. The receipt of triple therapy had the greatest impact on survival. DISCUSSION: This study highlights the survival benefit of a multimodal approach in the treatment of ATRT. The use of triple therapy, including surgery, radiation, and chemotherapy, was found to have the greatest survival benefit for patients. Overall, these findings may guide future care for patients with ATRT.
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Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Criança , Humanos , Pré-Escolar , Lactente , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/patologia , Teratoma/terapia , Teratoma/tratamento farmacológico , Tumor Rabdoide/patologia , Tumor Rabdoide/cirurgia , Terapia CombinadaRESUMO
OBJECTIVE: The natural history, treatment options, and clinical outcomes of pancreatic metastases to the brain remain largely unknown. Here, we seek to investigate characteristics that influence OS in pancreatic metastases to the brain. METHODS: This is a population-based retrospective study of OS in 508 patients with pancreatic metastases to the brain using the SEER database. Univariate and multivariate Cox regression analyses were utilized, and a predictive nomogram was developed. RESULTS: There were 508 patients identified for this study, with a median OS of 2 months. In the univariate analysis, patients older than 65 years had significantly reduced OS (P < 0.001). Patients with liver metastases (P < 0.001) and liver and lung metastases (P < 0.001) exhibited significantly reduced OS. Treatment of the primary tumor with chemotherapy only (P < 0.001), radiation only (P = 0.01), radiation and chemotherapy (P < 0.001), and surgery only (P = 0.01) were associated with increased OS. Resection of a distant metastasis site (P = 0.009) and of a brain metastasis (P = 0.03) were associated with increased OS. In the multivariable analysis, factors that remained significant included patient age (P = 0.01), liver metastases (P < 0.001), liver and lung metastases (P < 0.001), treatment with chemotherapy (P < 0.001), treatment with radiation and chemotherapy (P < 0.001), and treatment with surgery and chemotherapy (P < 0.001). The nomogram had a C-index of 0.766, suggesting congruence between the findings on the nomogram and the results in the internal verification. CONCLUSIONS: Median OS is influenced by age, multiorgan metastases, and treatment of the primary tumor. These data highlight the marginal benefit of treatment, yet improved quality of life (QOL) remains to be elucidated.
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Neoplasias Hepáticas , Neoplasias Pulmonares , Neoplasias Pancreáticas , Humanos , Prognóstico , Estudos Retrospectivos , Qualidade de Vida , Neoplasias Pancreáticas/terapia , Neoplasias Hepáticas/terapia , Neoplasias Pulmonares/terapia , Programa de SEERRESUMO
INTRODUCTION: Grade 3 solitary fibrous tumor, previously known as anaplastic hemangiopericytoma, is a rare and highly malignant intracranial tumor with a limited understanding of its natural history and treatment outcomes. METHODS: We conducted a retrospective analysis using the Surveillance, Epidemiology, and End Results (SEER) database spanning 2000-2019 to evaluate the clinical characteristics and treatment modalities that influence overall survival in this tumor entity. A cohort of 249 patients with intracranial grade 3 solitary fibrous tumors was identified. Univariate and multivariable Cox proportional hazard models were employed to determine significant prognostic factors for overall survival. Kaplan-Meier models were used to visualize survival curves, and a nomogram was constructed to predict survival probabilities at 6- and 12-month following diagnosis. RESULTS: Our findings indicated that patient age (<65 years), localized or regional disease burden, surgical resection, and radiation therapy were significant predictors of better overall survival. Combination therapies showed improved survival, with surgery and radiation therapy having the most significant impact. However, chemotherapy alone or in combination did not demonstrate a significant survival benefit, likely due to the limited sample size. The nomogram provided personalized prognostic predictions based on significant clinical factors. CONCLUSIONS: These data emphasize the importance of surgical resection and radiation therapy in the management of grade 3 solitary fibrous tumors, supporting the use of combination therapies to improve overall survival in this rare and aggressive intracranial neoplasm.
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Hemangiopericitoma , Programa de SEER , Tumores Fibrosos Solitários , Humanos , Estudos Retrospectivos , Masculino , Feminino , Tumores Fibrosos Solitários/terapia , Tumores Fibrosos Solitários/mortalidade , Tumores Fibrosos Solitários/patologia , Tumores Fibrosos Solitários/epidemiologia , Pessoa de Meia-Idade , Hemangiopericitoma/terapia , Hemangiopericitoma/mortalidade , Hemangiopericitoma/patologia , Hemangiopericitoma/epidemiologia , Idoso , Prognóstico , Adulto , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/epidemiologia , Nomogramas , Gradação de Tumores , Estimativa de Kaplan-Meier , Adulto Jovem , Idoso de 80 Anos ou mais , Terapia CombinadaRESUMO
BACKGROUND: Methotrexate is an immunosuppressant commonly used to treat inflammatory conditions, such as rheumatoid arthritis. However, albeit exceedingly rare, it can have serious adverse effects within the central nervous system (CNS), such as methotrexate-associated lymphoproliferative disorder (MTX-LPD). Literature describing the natural history, treatment options, and clinical outcomes of patients with CNS MTX-LPD remains sparse. METHODS: We present a systematic literature review following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and a case illustration of CNS MTX-LPD. RESULTS: A systematic review of the literature revealed 12 published cases of CNS MTX-LPD, plus the case presented herein, for a total of 13 included cases. The most common indication for MTX was rheumatoid arthritis. The most common treatment for the LPD was MTX cessation (12, 92.3%), adjunct chemotherapy (2, 15.4%), total tumor resection (3, 23.1%), or steroid therapy (1, 7.7%). Treatment usually led to improvement of neurological symptoms (9, 69.2%) along with regression of the lesions (3, 23.1%) with no recurrence (6, 46.2%). Death was reported in four cases (30.8%) with a mean time from onset of 11 months. CONCLUSIONS: CNS MTX-LPD should be considered in the differential diagnosis for patients who are taking MTX presenting with neurologic symptoms, as immediate withdrawal of MTX has demonstrated good prognosis.
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Artrite Reumatoide , Doenças do Sistema Nervoso Central , Transtornos Linfoproliferativos , Humanos , Metotrexato/efeitos adversos , Artrite Reumatoide/tratamento farmacológico , Imunossupressores/efeitos adversos , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/tratamento farmacológico , Doenças do Sistema Nervoso Central/complicações , Sistema Nervoso CentralRESUMO
BACKGROUND: We describe the first-in-human experience using the Balloon Occlusion Stroke Sheath (BOSSTM) balloon-guide catheter to perform stroke thrombectomy in 50 consecutive patients enrolled in the Flow Arrest Safety and Technical success with balloon-guide catheters trial. This aspiration system includes a novel 9.4F balloon-guide catheter conduit for the insertion and guidance of catheters with a balloon providing temporary flow arrest. METHODS: The Flow Arrest Safety and Technical success with balloon-guide catheter trial is a single-arm, prospective, multi-center, non-randomized, observational registry evaluating the use of the market-released BOSSTM balloon-guide catheter in adult patients diagnosed with an acute ischemic stroke attributable to large vessel occlusion. The purpose of the current trial was to assess the safety and technical success associated with the use of the BOSSTM balloon-guide catheter. RESULTS: Fifty patients met inclusion criteria with a mean baseline National Institutes of Health Stroke Scale (NIHSS) of 16. Treatment devices, including aspiration and stent retriever devices, were used in a total of 88 passes. The BOSSTM balloon-guide catheter was compatible with all stroke thrombectomy treatment devices used in 98% (49/50) of procedures. Balloon inflation and flow arrest were achieved in 100% (50/50) and 98% (49/50) of cases, respectively. Balloon deflation and retraction were observed in 100% (50/50) of cases. Successful reperfusion (modified thrombolysis in cerebral infarction score > 2b) was achieved in 100% of cases with single-pass reperfusion achieved in 62% (31/50) of cases. CONCLUSIONS: The BOSSTM balloon-guide catheter is a safe and technically effective adjunctive device for mechanical thrombectomy of acute ischemic stroke due to large vessel occlusion.
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BACKGROUND: Diffuse midline glioma with histone H3K27M mutation (H3K27M DMG) is a recently recognized World Health Organization grade IV glioma with a dismal prognosis. Despite maximal treatment, this high-grade glioma exhibits an estimated median survival of 9-12 months. However, little is known with regards to prognostic risk factors for overall survival (OS) for patients with this malignant tumor. The aim of the present study is to characterize risk factors influencing survival in H3K27M DMG. METHODS: This is a population-based retrospective study of survival in patients with H3K27M DMG. The Surveillance, Epidemiology, and End Results database was examined from the years 2018 to 2019 and data from 137 patients were collected. Basic demographics, tumor site, and treatments regimens were retrieved. Univariate and multivariable analyses were conducted to assess for factors associated with OS. Nomograms were built based on the results of the multivariable analyses. RESULTS: Median OS of the entire cohort was 13 months. Patients with infratentorial H3K27M DMG exhibited worse OS compared to their supratentorial counterparts. Any form of radiation treatment resulted in a significantly improved OS. Most combination treatments significantly improved OS with the exception of the surgery plus chemotherapy group. The combination of surgery and radiation had the greatest impact on OS. CONCLUSIONS: Overall, the infratentorial location of H3K27M DMG portends a worse prognosis compared to their supratentorial counterparts. The combination of surgery and radiation had the greatest impact on OS. These data highlight the survival benefit in utilizing a multimodal treatment approach for H3K27M DMG.
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Neoplasias Encefálicas , Glioma , Humanos , Prognóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Estudos Retrospectivos , Glioma/genética , Glioma/terapia , Histonas/genética , Mutação/genéticaRESUMO
BACKGROUND: Pure arterial malformations are characterized as unique cerebrovascular lesions with a dilated, coil-like appearance and tortuous arteries without early venous drainage. Historically, these lesions have been described as incidental findings with a benign natural history. However, pure arterial malformations can rarely demonstrate radiographic progression and develop associated focal aneurysms with an unclear risk of rupture. Whether radiographic progression of these lesions or the presence of an associated aneurysm warrants treatment remains controversial. OBSERVATIONS: A 58-year-old male presented with sudden-onset left hemiparesis. Computed tomography revealed a large, acute, right frontotemporoparietal intraparenchymal hemorrhage with underlying irregular curvilinear calcifications. Diagnostic cerebral angiography revealed a dysplastic right middle cerebral artery dissecting aneurysm along the M2 segment associated with a pure arterial malformation, which was treated with endovascular flow diversion in a delayed fashion. LESSONS: Pure arterial malformations with associated focal aneurysms may not exhibit a benign natural history as once thought. Intervention should be considered for ruptured pure arterial malformations to mitigate the risk of rerupture. Asymptomatic patients with a pure arterial malformation with an associated aneurysm should at least be followed closely with interval radiographic imaging to evaluate for malformation progression or changes in aneurysmal morphology.
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Diffuse hemispheric glioma, H3 G34-mutant, is a recently recognized distinct high-grade glioma with a dismal prognosis. In addition to the H3 G34 missense mutation, numerous genetic events have been identified in these malignant tumors, including ATRX, TP53, and, rarely, BRAF genes. There are only a few reports to date that have identified BRAF mutations in diffuse hemispheric glioma, H3 G34-mutant. Moreover, to our knowledge, gains of the BRAF locus have yet to be described. Here, we present a case of an 11-year-old male with a diffuse hemispheric glioma, H3 G34-mutant, found to have novel gains of the BRAF locus. Furthermore, we emphasize the current genetic landscape of diffuse hemispheric glioma, H3 G34-mutant, and implications of an aberrant BRAF signaling pathway.
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OBJECTIVE: The authors sought to investigate the association between white blood cell counts and acute hydrocephalus in spontaneous nonaneurysmal subarachnoid hemorrhage (nSAH). METHODS: We conducted a retrospective analysis of 105 consecutive patients with spontaneous nSAH. Univariate and multivariable logistic regression analyses were performed to investigate factors associated with hydrocephalus. Receiver operating characteristic curve analysis determined the optimal cutoff to differentiate between patients with and without hydrocephalus. The admission characteristics of hydrocephalic patients with aneurysmal and nSAH were compared. RESULTS: A total of 70 patients met inclusion criteria, of which 21 (30%) presented with hydrocephalus. In univariate logistic regression, leukocytes, neutrophils, lymphocytes, neutrophil-to-lymphocyte ratio, lymphocyte-to-monocyte ratio, neutrophil-monocyte-to-lymphocyte ratio, and the systemic immune-inflammation (SII) index ([neutrophils × platelets/lymphocytes]/1000) were associated with hydrocephalus. After adjustments, the SII index independently predicted acute hydrocephalus with the highest odds among laboratory values (odds ratio 2.184, P = 0.006). Receiver operating characteristic curve analysis revealed the SII index differentiated between patients with and without hydrocephalus (area under the curve = 0.799, 95% CI: 0.688-0.909, P < 0.001) with an optimal cutoff of 1.385 103/µL. SII indices did not differ between aneurysmal and nSAH patients with hydrocephalus (3.5 vs. 3.6 103/µL, P = 0.795). CONCLUSIONS: A SII index ≥1.385 103/µL on admission predicts acute hydrocephalus in spontaneous nSAH. Hydrocephalic patients with aneurysmal and nSAH exhibit similar SII indices, and thus, an exaggerated inflammatory and thrombotic response follows spontaneous subarachnoid hemorrhage irrespective of hemorrhage etiology.
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Hidrocefalia , Hemorragia Subaracnóidea , Humanos , Hemorragia Subaracnóidea/complicações , Estudos Retrospectivos , Inflamação/complicações , Linfócitos , Hidrocefalia/complicações , PrognósticoRESUMO
BACKGROUND: Adult spinal intradural arachnoid cysts are rare pathologic entities with an unclear etiopathogenesis. These lesions can be dichotomized into primary (idiopathic) or secondary (related to inflammation, intradural surgery, or trauma) etiologies. Limited series have depicted optimal management strategies and clinical outcomes. OBJECTIVE: To illustrate our experience with spinal intradural arachnoid cysts and to present a literature review of surgically treated cysts to elucidate the clinical and anatomic differences between etiologies. METHODS: Institutional review revealed 29 patients. Various data were extracted from the medical record. Initial and follow-up symptomatologies of the surgical cohort were compared. The literature review included case series describing cysts managed surgically. RESULTS: From patients treated surgically at our institution (22), there was a significant reduction in thoracic back pain postoperatively ( P = .034). A literature review yielded 271 additional cases. Overall, primary and secondary lesions accounted for 254 and 39 cases, respectively. Cysts of secondary origin were more likely localized ventral to the spinal cord ( P = .013). The rate of symptomatic improvement after surgical intervention for primary cysts was more than double than that of secondary cysts ( P < .001). Compared with primary etiologies, the rates of radiographic progression ( P = .032) and repeat surgery ( P = .041) were each more than double for secondary cysts. CONCLUSION: Surgical intervention for spinal intradural arachnoid cysts improves thoracic back pain. The literature supports surgical intervention for symptomatic primary spinal intradural arachnoid cysts with improved clinical outcomes. Surgery should be cautiously considered for secondary cysts given worse outcomes.
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Cistos Aracnóideos , Doenças da Medula Espinal , Humanos , Adulto , Doenças da Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/etiologia , Doenças da Medula Espinal/cirurgia , Cistos Aracnóideos/diagnóstico por imagem , Cistos Aracnóideos/cirurgia , Dor nas Costas/etiologia , Dor nas Costas/cirurgia , Imageamento por Ressonância Magnética/efeitos adversosRESUMO
BACKGROUND: Depressed skull fractures are typically the consequence of high-impact injuries with inward buckling of the cranium. The majority of depressed skull fractures are managed conservatively in the absence of dural violation, sinus involvement, significant underlying hematoma, depressed fragment greater than 1 cm, wound infection, or gross wound contamination. Even in the presence of any of the aforementioned criteria, cranioplasty is typically considered an urgent procedure rather than a neurosurgical emergency. Rarely, a depressed fracture fragment can cause focal neurologic deficit(s) due to direct compression of the underlying eloquent cortex. CASE DESCRIPTION: A 40-year-old male presented to the emergency department after a mechanical fall with a left central facial nerve palsy, left hemiplegia, left hemianesthesia, and fixed right gaze deviation. The neurologic deficits observed were attributed to a combination of blunt force trauma to the head (i.e., coup-contrecoup injury) and the depressed fracture fragment compressing the underlying eloquent cortex. He underwent emergent cranioplasty with fragment elevation within 2 hours of the traumatic injury. At 6-month follow-up, he regained full neurologic function without any residual deficits. CONCLUSIONS: Our experience highlights a rare indication for emergent cranioplasty with an excellent functional outcome attributable to immediate fracture elevation and decompression of eloquent cortex.
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Fratura do Crânio com Afundamento , Fraturas Cranianas , Ferimentos não Penetrantes , Masculino , Humanos , Adulto , Fratura do Crânio com Afundamento/complicações , Fratura do Crânio com Afundamento/diagnóstico por imagem , Fraturas Cranianas/cirurgia , HematomaRESUMO
Encephaloceles rarely develop following traumatic skull fractures. Given their low incidence, the clinical presentations and management strategies of these lesions are confined to case reports and limited case series. A systematic literature review was performed using PubMed, Ovid, and Web of Science databases to identify relevant articles using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A total of 37 articles met inclusion criteria, including the case presented herein. These articles reported 52 traumatic encephaloceles. Mean patient age was 25.3 years (range 6 mo-66 y) with a male predominance (63%, 33/52). The most common bony defects resulting in encephalocele formation were the orbital roof (52%, 27/52), ethmoid (35%, 18/52), and sphenoid (10%, 5/52). Mean time from traumatic injury to initial presentation was 21.3 months (range 0 d-36 y) with a bimodal distribution split between immediately following the traumatic injury (57%, 26/46) or in a delayed manner (43%, 20/46). Common presentations of orbital roof, frontonasal, and temporal bone encephaloceles were exophthalmos (85%, 23/27), cerebrospinal fluid rhinorrhea (71%, 17/24), and hearing loss (100%, 4/4), respectively. Operative approach, repair technique, and materials used for encephalocele reduction were highly variable. Surgical intervention afforded definitive symptomatic improvement or resolution in the majority of cases (89%, 42/47). Clinical outcomes did not differ between orbital, frontonasal, or temporal bone encephaloceles ( P =0.438). Traumatic encephaloceles are a rare entity with diverse presenting symptomatology dependent upon the location of fracture dehiscence. Surgical intervention affords symptomatic improvement in the majority of cases irrespective of encephalocele location, time to presentation, or operative approach.
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Rinorreia de Líquido Cefalorraquidiano , Perda Auditiva , Humanos , Masculino , Criança , Feminino , Encefalocele/diagnóstico por imagem , Encefalocele/etiologia , Encefalocele/cirurgia , Osso Temporal/patologia , Órbita/patologia , Perda Auditiva/complicaçõesRESUMO
Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized C11orf95-RELA fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with C11orf95-RELA fusions and supratentorial ependymomas with C11orf95 RELA fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of RELA fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with C11orf95-RELA fusions should be classified as a distinct entity.
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The authors sought to evaluate whether immunologic counts on admission were associated with shunt-dependent hydrocephalus following aneurysmal subarachnoid hemorrhage. A retrospective analysis of 143 consecutive patients with aneurysmal subarachnoid hemorrhage over a 9-year period was performed. A stepwise algorithm was followed for external ventricular drain weaning and determining the necessity of shunt placement. Data were compared between patients with and without shunt-dependent hydrocephalus. Overall, 11.19% of the cohort developed shunt-dependent hydrocephalus. On multivariate logistic regression analysis, acute hydrocephalus (OR: 61.027, 95% CI: 3.890-957.327; p = 0.003) and monocyte count on admission (OR: 3.362, 95% CI: 1.024-11.037; p = 0.046) were found to be independent predictors for shunt dependence. Receiver operating characteristic curve analysis for the prediction of shunt-dependent hydrocephalus confirmed that monocyte count exhibited an acceptable area under the curve (AUC = 0.737, 95% CI: 0.601-0.872; p < 0.001). The best predictive cutoff value to discriminate between successful external ventricular drain weaning and shunt-dependent hydrocephalus was identified as a monocyte count ≥0.80 × 103/uL at initial presentation. These preliminary data demonstrate that a monocyte count ≥0.80 × 103/uL at admission predicts shunt-dependent hydrocephalus in patients with aneurysmal subarachnoid hemorrhage; however, further large-scale prospective trials and validation are necessary to confirm these findings.
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Metastasis of ovarian carcinoma to the central nervous system occurs in <2% of cases and classically localizes within the brain parenchyma. Moreover, leptomeningeal spread of these tumors is an exceedingly rare phenomenon. Here, we conduct a systematic review of the current literature on the natural history, treatment options, and proposed pathogenic mechanisms of leptomeningeal carcinomatosis in ovarian carcinoma. We also report a case of a 67-year-old female with stage IV metastatic ovarian serous carcinoma initially confined to the peritoneal cavity with a stable disease burden over the course of three years. Follow-up imaging demonstrated an intracranial lesion, which was resected via craniotomy, and pathology was consistent with the original diagnosis. Three months after surgery, she developed rapidly progressive dizziness, generalized weakness, fatigue, and ataxia. Repeat MRI demonstrated interval development of extensive and diffusely enhancing dural nodularity, numerous avidly enhancing supratentorial and infratentorial lesions, enhancement of the bilateral trigeminal nerves, internal auditory canals, and exit wound from the surgical site into the posterior aspect of the right-sided neck musculature consistent with diffuse leptomeningeal dissemination. The present case highlights that leptomeningeal dissemination of ovarian carcinoma is a potential yet rare consequence following surgical resection of an ovarian parenchymal metastasis. Progressive clinical symptomatology that develops postoperatively in this patient population should prompt urgent workup to rule out leptomeningeal disease and an expedited radiation oncology consultation if identified.
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BACKGROUND: Neurenteric cysts (NC) are uncommon congenital lesions with histopathologic properties derived from the gastrointestinal or respiratory tract. They are typically located in the intradural extramedullary compartment but rarely seen in the supratentorial region. The occurrence of supratentorial NCs (S-NC) presents an interesting quandary regarding their embryopathogenesis. METHODS: We present a case report and systematic literature review on S-NCs following PRISMA guidelines. RESULTS: A 57-year-old woman presented with a seizure and paresthesias of the face, hands, and feet. Magnetic resonance imaging showed a right temporo-occipital cystic lesion, which was managed with surgical resection. Histologically, the cyst was type A. The patient was without recurrence at 10 months. Including this case, 88 S-NCs have been reported in the literature. Common presenting symptoms are headaches and seizures. They were mostly treated with craniotomy, preferably with gross total resection, although subtotal resection may be necessary because of adhesions. Resection usually led to symptom improvement (61%). Malignant transformation was seen in 3%. Recurrence was seen in 17%, with a mean time to recurrence of 4.2 years, and was significantly more common after subtotal resection than gross total resection. CONCLUSIONS: If surgically resected, the cyst wall specimen should be sent for pathology review, because of the potential risk for malignancy. If conservatively managed, serial imaging is warranted to track for changes that may indicate transformation. The embryopathogenesis of these rare congenital lesions remains incompletely understood, but the most comprehensive theory involves enteric cell migration to the neuroectoderm during embryogenesis.
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Cistos , Defeitos do Tubo Neural , Craniotomia/efeitos adversos , Cistos/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Pessoa de Meia-Idade , Defeitos do Tubo Neural/diagnóstico por imagem , Defeitos do Tubo Neural/cirurgia , Convulsões/etiologiaRESUMO
OBJECTIVE: The authors sought to investigate if peripheral blood leukocyte profiles on admission differed between perimesencephalic, angio-occult, and aneurysmal subarachnoid hemorrhage cohorts. METHODS: We performed a retrospective analysis of 202 consecutive patients with spontaneous subarachnoid hemorrhage. We classified spontaneous subarachnoid hemorrhage as either aneurysmal or nonaneurysmal origin. Nonaneurysmal subarachnoid hemorrhage was subclassified as either perimesencephalic or angio-occult according to the distribution of hemorrhage on the initial imaging. Patient demographics, clinical parameters, radiographic metrics, and laboratory values were obtained on admission. In-hospital data including acute hydrocephalus, shunt dependence, vasospasm, and delayed cerebral ischemia were collected. Comparative analyses were conducted between cohorts. RESULTS: The perimesencephalic subarachnoid hemorrhage cohort exhibited significantly lower neutrophil (7.76 vs. 10.06; P = 0.004), lymphocyte (1.40 vs. 1.90; P = 0.024), and monocyte counts (0.52 vs. 0.73; P = 0.031) than the aneurysmal subarachnoid hemorrhage cohort. There were no significant differences in peripheral blood leukocyte profiles between the angio-occult and aneurysmal subarachnoid hemorrhage cohorts. The nonaneurysmal cohort exhibited significantly lower neutrophil (8.33 vs. 10.06; P = 0.005) and lymphocyte counts (1.47 vs. 1.90; P = 0.011) as well as a lower lymphocyte-to-monocyte ratio (2.80 vs. 4.51; P = 0.018) than the aneurysmal subarachnoid hemorrhage cohort. CONCLUSIONS: Perimesencephalic subarachnoid hemorrhage exhibits a unique peripheral blood leukocyte profile compared to aneurysmal subarachnoid hemorrhage. Moreover, these preliminary data demonstrate that blood leukocytes may be affected by the burden of cisternal subarachnoid hemorrhage or the presence of a ruptured aneurysm. Further large-scale prospective studies and validation are required to confirm these preliminary findings.
Assuntos
Hemorragia Subaracnóidea , Humanos , Leucócitos , Estudos Prospectivos , Estudos Retrospectivos , Hemorragia Subaracnóidea/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Cerebral amebic encephalitis due to Balamuthia mandrillaris is a rare yet typically fatal disease. As such, identification of the clinical characteristics, appropriate diagnostic workup and commencement of treatment is frequently delayed. Here, we present a case of a 4-year-old male with a B. mandrillaris cerebral abscess successfully treated with expedited neurosurgical resection and broad-spectrum antimicrobial therapy.