Natural history and neuro-oncological approach in spinal gangliogliomas: a systematic review.

To describe the natural history of spinal gangliogliomas (GG) in order to determine the most appropriate neuro-oncological management. A Medline search for relevant publications up to July 2023 using the key phrase "ganglioglioma spinal" and "ganglioglioma posterior fossa" led to the retrieval of 178 studies. This corpus provided the basis for the present review. As an initial selection step, the following inclusion criteria were adopted: (i) series and case reports on spinal GG; (ii) clinical outcomes were reported specifically for GG; (iii) GG was the only pathological diagnosis for the evaluation of the tumor; (iv) papers written only in English was evaluated; and (v) papers describing each case in the series were included. The World Health Organization (WHO) 2021 grading criteria for gangliogliomas were applied. A total of 107 tumors were evaluated (63 from male patients and 44 from female patients; 1.43 male/1.0 female ratio, mean age 18.34 ± 15.84 years). The most common site was the cervical spine, accounting for 43 cases (40.18%); GTR was performed in 35 cases (32.71%) and STR in 71 cases (66.35%), while this information was not reported in 1 case (0.94%). 8 deaths were reported (7.47%) involving 2 males (25%) and 6 females (75%) aged 4-78 years (mean 34.27 ± 18.22) years. GGs located on the spine displayed the same gender ratio as these tumors in general. The most frequent symptom was pain and motor impairment, while the most prevalent location was the cervical spinal cord. GTR of the tumor posed a challenge for neurosurgeons, due to the difficulty of resecting the lesion without damaging the spinal eloquent area, explaining the lower rate of cure for this tumor type.

Impact of a cell cycle and an extracellular matrix remodeling transcriptional signature on tumor progression and correlation with EZH2 expression in meningioma.

OBJECTIVE: The authors searched for genetic and transcriptional signatures associated with tumor progression and recurrence in their cohort of patients with meningiomas, combining the analysis of targeted exome, NF2-LOH, transcriptome, and protein expressions. METHODS: The authors included 91 patients who underwent resection of intracranial meningioma at their institution between June 2000 and November 2007. The search of somatic mutations was performed by Next Generation Sequencing through a customized panel and multiplex ligation-dependent probe amplification for NF2 loss of heterozygosity. The transcriptomic profile was analyzed by QuantSeq 3' mRNA-Seq. The differentially expressed genes of interest were validated at the protein level analysis by immunohistochemistry. RESULTS: The transcriptomic analysis identified an upregulated set of genes related to metabolism and cell cycle and downregulated genes related to immune response and extracellular matrix remodeling in grade 2 (atypical) meningiomas, with a significant difference in recurrent compared with nonrecurrent cases. EZH2 nuclear positivity associated with grade 2, particularly with recurrent tumors and EZH2 gene expression level, correlated positively with the expression of genes related to cell cycle and negatively to genes related to immune response and regulation of cell motility. CONCLUSIONS: The authors identified modules of dysregulated genes in grade 2 meningiomas related to the activation of oxidative metabolism, cell division, cell motility due to extracellular remodeling, and immune evasion that were predictive of survival and exhibited significant correlations with EZH2 expression.

Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients / Impacto das características angioarquiteturais das malformações arteriovenosas cerebrais na apresentaçáo clínica: análise de 183 pacientes

ABSTRACT Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

RESUMO Antecedentes: A correlação entre a angioarquitetura e a apresentação clínica da Malformação Arteriovenosa do cérebro (MAVc) permanece um assunto de debate. Objetivos: Correlacionar as características angioarquiteturais das MAVc com a apresentação clínica. Métodos: Estudou-se pacientes consecutivos atendidos no Hospital Beneficência Portuguesa-SP, entre 2006 a 2016. Após análise geral, criaram-se cinco grupos de acordo com a apresentação clínica: 1- Hemorragia; 2 - Epilepsia; 3 - Cefaléia; 4 - Déficit Neurológico Progressivo (DNP) e 5 - Incidental. Características epidemiológicas (clínica e topografia) e angioarquiteturais (Classificação de Spetzler-Martin Modificada - SMM; Fluxo intranidal; Aneurismas arteriais, intranidais e venosos; Ectasia venosa; Congestão venosa; "Roubo" arterial; Vascularização dural; Drenagem Venosa Profunda) foram analisadas. Resultados: 183 pacientes foram incluídos e analisados globalmente. Após essa etapa, foram divididos nos grupos: 1 - 56 casos (30,6%); 2 - 49 casos (26,7%); 3 - 41 casos (22,4%); 4 - 28 casos (15,3%) e 5 - 9 casos (4,9%). Principais achados foram referentes a apresentação hemorrágica, na qual observamos correlação estatística positiva com o sexo feminino (P<0,02), lesões classificadas como SMM 3B (P<0,0015) e baixo fluxo (P<0,04). Relacionado à epilepsia, encontramos significância estatística que possibilitou a correlação com pacientes com idade inferior a 36 anos (P<0,001), sexo masculino (P<0,018), lesões superficiais (P<0,002), presença de ectasia venosa (P<0,003) e "roubo" arterial (P<0,01). Pacientes com DNP se apresentam com idade superior aos demais (P<0,01). Conclusões: Após análise multivariada, foi possível separar as MAV em grupos de acordo com as características angioarquiteturais, comprovando que algumas dessas características estão fortemente relacionadas a determinada manifestação.

Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients.

BACKGROUND: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. OBJECTIVE: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. METHODS: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). RESULTS: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). CONCLUSIONS: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

Assessment of hemorrhagic onset on meningiomas: Systematic review.

OBJECTIVE: To review the data published on the subject to create a more comprehensive natural history of the haemorrhagic onset of meningiomas (IVMs). PATIENTS AND METHODS: A Medline search up to June 2020, using the search term "bleeding meningioma," returned 136 papers. As a first selection step, we adopted the following inclusion criteria: series and case reports about bleeding meningioma. Papers written in other languages but with abstracts written in English were also evaluated. RESULTS: A total of 190 tumours were evaluated, specifically 109 tumours from female patients and 81 tumours from male patients with a ratio of 1.34 female to 1.0 male (mean age of 54.86 ± 16.1years old). The majority were located in the convexity (129-67.9 %). Among the 190 tumours evaluated, 171 patients (90 %) presented with GI tumours, with a predominance of the meningothelial subtype (32.6 %). Nine patients (4.7 %) presented with grade GII tumours, and 10 (5.3 %) presented with GIII tumours. The most prevalent type was intracerebral haemorrhage (ICH) at 50 %, followed by subdural at 27.36 %; the mortality rate was 13.1 % (25 deaths), the distribution of both location (prevalence of convexity: 18-72 %) and histopathology (grade 1: 22-88 %). CONCLUSION: These tumours follow the histopathological distribution of meningiomas, in general. The age distribution shows prevalence among the adult population but with a greater proportion in the elderly. The fact that the overwhelming majority of cases involve meningiomas with a benign histological subtype is noteworthy. Another relevant factor observed is that most reports are from Asian origin.

Spontaneous Convexity Subarachnoid Hemorrhage Caused by Internal Carotid Occlusion: Radiological Features

A 79-year-old patient was admitted to the emergency room with transitory monoparesis in the left hand and dysphasia. The brain computed tomography (CT) and magnetic resonance imaging (MRI) showed a spontaneous right convexity subarachnoid hemorrhage (cSAH). Digital subtraction angiography (DSA) confirmed an asymptomatic occlusion of the right internal carotid artery (ICA) . Cases related to stenosis have already been described, but there is no similar report of a case related to occlusion, even though the pathophysiology of both entities is similar. Atraumatic SAH has been associated with intracranial and extracranial artery stenosis.

Cyclin E1 expression and malignancy in meningiomas.

OBJECTIVE: The aim of the present study was to analyze if the pathway Skp2-p27-cyclin E1 could also be a tumor progression marker for meningiomas. PATIENTS AND METHODS: We used quantitative real-time PCR to assess the relative expression levels of the genes coding for cyclin E1 (CCNE1), Skp2 (SKP2), and p27 (P27). The expression levels were compared in grades I to III meningiomas and among different histological subtypes of grade I meningiomas. RESULTS: Anaplastic meningiomas accounted for 4.9%, atypical meningiomas for 23.5% and grade I meningiomas for 71.6%.CCNE1 expression level was significantly higher in grade II compared to grade I meningiomas (p = 0.0027), and its expression level reliably predicts grade II meningiomas (ROC AUC = 0.731, p = 0.003). CCNE1 expression also correlated with SKP2 and P27 expression levels in grade I meningiomas (r = 0.539, p < 0.0001 and r = 0.687, p = <0.0001, respectively for CCNE1/SKP2 and CCNE1/P27, Spearman's test). Fibrous subtype among grade I meningiomas presented the highest expression levels of CCNE1, SKP2 and P27. Higher expression of cyclin E1 protein was detected in the nuclei of atypical meningiomas compared to grade I meningiomas. CONCLUSIONS: CCNE1 expression level predicts meningioma malignancy, and the fibrous subtype presents the highest gene expression levels among grade I meningiomas.

Natural history of intraventricular meningiomas: systematic review.

Review the data published on the subject to create a more comprehensive natural history of intraventricular meningiomas (IVMs). A Medline search up to March 2018 using "intraventricular meningioma" returned 98 papers. As a first selection step, we adopted the following inclusion criteria: series and case reports about IVMs, as well as papers written in other languages, but abstracts written in English were evaluated. Six hundred eighty-one tumors were evaluated from 98 papers. The majority of the tumors were located in the lateral ventricles (602-88.4%), fourth ventricle (59-8.7%), and third ventricle (20-2.9%). These tumors accounted for a mortality rate of 4.0% (25 deaths) and a recurrence rate of 5.3% (26 recurrences). The majority of the tumors were grade I (89.8%) and consisted of the following subtypes: fibrous, 39.7% (n = 171); transitional, 22.0% (n = 95); meningothelial, 18.6% (n = 80); angiomatosus, 3.2% (n = 14); psammomatous, 2.6% (n = 11); and others, 13.9% (n = 60). Forty-five patients (7.4%) presented with grade II (GII) tumors, and 17 patients (2.8%) presented with grade III (GIII) tumors. These tumors follow the histopathological distribution of meningiomas in general, with the exception of the higher prevalence of the fibrous subtype, possibly due to its embryonic origin. Recurrence and mortality were lower than in other localizations likely due to a complete surgical resection rate than in the convexity and skull base, which suggests that GTR is the gold standard for the management of IVMs.

Impact of radiotherapy in atypical meningioma recurrence: literature review.

Evaluate whether radiotherapy (RT) after the neurosurgical treatment of atypical meningiomas (AM) has an impact on the reduction rate of recurrence. A Medline search through October 2017 using "atypical meningioma" returned 1277 papers for initial review. Inclusion criteria were as follows. We analyzed the database and included articles in which the anatomic pathological classification of atypical meningiomas was in accordance with WHO 2007 or WHO 2016 criteria, patients > 18 years of age, and there was postoperative external beam radiation to the tumor bed. Exclusion criteria were WHO grade I or III meningioma, patients who underwent whole-brain radiation, RT used as salvage therapy for recurrence, palliative dose of RT (< 45 Gy), recurrent AMs, and multiple AMs. Papers reporting outcomes in which atypical and anaplastic meningiomas were analyzed together were rejected, as were papers with small samples that may compromise evaluation. After filtering our initial selection, only 17 papers were selected. After reviewing the seventeen articles including a total of 1761 patients (972 female and 799 male; 1.21 female/1.0 male), the difference in proportion of tumor recurrence between patients with and without radiotherapy after neurosurgical procedure was 1.0448, 95% CI [0.8318 to 1.3125], p value = 0.7062. On the basis of this review, there is no evidence to suggest that RT decreases the rate of recurrence in patients with atypical meningiomas.

Molecular alterations in meningiomas: Literature review.

Meningiomas, tumors that originate from meningothelial cells, account for approximately 30% of all new diagnoses of central nervous system neoplasms. According to the 2016 WHO classification of central nervous system tumors meningiomas are classified into three grades: I, II, and III. Past studies have shown that the risk of meningiomas recurrence is strongly correlated with the molecular profile of the tumor. Extensive whole-exome or whole-genome sequencing has provided a large body of information about the mutational landscape of meningiomas. However, such a stratification of meningiomas based on mutational analysis alone has been proven not to satisfy the clinical need for distinction between patients who need (or do not need) an adjuvant treatment. Combined analysis of exome, transcriptome, methylome and future approaches for epigenetic aspects in meningiomas may allow researchers to unveil a more comprehensive understanding of tumor progression mechanisms and, consequently, a more personalized clinical approach for patients with meningioma. A better understanding of the genetics and clinical behavior of high-grade meningiomas is mandatory in order to better design future clinical trials. By studying the mechanisms underlying these new tumorigenesis pathways, we should be able to offer personalized chemotherapy to patients with surgery and radiation-refractory meningiomas in the near future. The purpose of this article is to accurately bring the compilation of this information, for a greater understanding of the subject.

Atypical and Malignant Meningiomas: Neurooncologic Management in a Brazilian cohort.

OBJECTIVE: To analyze the surgical and oncologic treatment of a Brazilian cohort of patients with grade II and III meningioma at a follow-up time of 15 years to get an overview of these patients' outcomes. METHODS: Cross-sectional study of 43 patients (26 women, 17 men; age range 20 to 83 years; average 57.72 ± 14.54) operated on from 2000 to 2014 at a single institution, with the neuropathologic diagnosis of meningioma grade II (39 patients) and grade III (4 patients). RESULTS: Radiotherapy: 24 patients (55.81%) underwent radiotherapy; the time between the surgical procedure and the beginning of radiotherapy was 5 months; 7 patients with a diagnosis of AM underwent a new surgical procedure, albeit of adjuvant therapy, because of tumor recurrence, and only 3 of them underwent radiotherapy after the first resection. Mortality: in total, 19 deaths (44.18%) were identified in this sample: 15 (38.46%) with GII and 4 (100%) with GIII. The 10-year survival was expected in 35% of GII patients and 0% of GIII patients. CONCLUSION: Surgery is still the main form of treatment and the mainstay for prolonging survival. Radiotherapy is still controversial; however, we observed its positive impact on recurrence and progression-free survival.

Clinical Outcome, Tumor Recurrence, and Causes of Death: A Long-Term Follow-Up of Surgically Treated Meningiomas.

OBJECTIVE: The medical literature still lacks information about the impact of surgery and adjuvant treatment on the life of patients with meningioma. The clinical outcome, timing of tumor recurrence, and causes of death are often overlooked. This study evaluates these data taking into account tumor localization and histologic grade. METHODS: The article is a cross-sectional study of patients operated on between 2000 and 2014 in a single institution. The series has 593 adult patients (442 females and 151 males) and follow-up of 68.8 ± 48.9 months. Imaging of 434 patients was reviewed and 379 patients/families interviewed. RESULTS: Sixty-eight deaths were related to tumor treatment/progression and 36 to other causes. After 2 years of surgery, deaths not related to tumor were 7 times more frequent than were tumor-related deaths (odds ratio, 7.1; 95% confidence interval, 2.8-19.5; P < 0.0001). Ten-year survival was expected in 85% of patients with grade I (GI) meningioma, 35% of patients with atypic (GII) meningioma, and 0% of patients with anaplastic (GIII) meningioma. Convexity tumors had about half the risk of recurrence compared with other localizations (odds ratio, 0.4; 95% confidence interval, 0.27-0.67; P = 0.0002). In GI meningioma, recurrence was neither related to death nor to impairment of independent life. All patients with GII and GIII meningioma who had recurrence died. 96.3% of interviewees reported neurologic improvement or stability after the surgery. CONCLUSIONS: Histologic grade is the most important factor for long-term survival. Complete resection has to be pursued in GII and GIII meningioma but must be carefully weighed against morbidities in GI meningioma.

Intracranial aneurysm arising from infundibular dilation.

Infundibular dilations (IDs) are funnel-shaped enlargements in the origin of intracranial arteries. Usually, IDs occur at the junction between posterior communicating artery (PcomA) and internal carotid artery (ICA). Progression from an ID of the PcomA to aneurysms has been described, but is unclear whether an ID is a preaneurysmal state or a normal anatomical variant. The authors describe a 55-year-old female patient presenting multiple intracranial aneurysms with a small one arising from an ID at the level of posterior communicating segment of ICA. To the best of our knowledge, after a criterious search in the English literature, it is the first description of such unusual combination. The preoperative neurosurgical planning is discussed as well as the importance of intraoperative findings to the best management of this unusual situation.

Paraplegia espástica causada por cisticercose intramedular: apresentação de caso e breve revisão bibliográfica / Spastic paraplegia due to spinal cord cisticercosis: case report

Relato de cisticercose intramedular em homem de 42 anos que há seis anos sentia parestesias em ambas as pernas, que evoluíram para ausência de sensação dolorosa, por fim paraparesia espástica dos membros inferiores. Ao exame físico, verificaram-se sinal de Babinski bilateral, espasticidade dos membros inferiores e déficit de sensibilidade abaixo de T7. Ressonância nuclear magnética mostrou uma lesão expansiva ao nível de T5. Durante a exérese do processo, constatou-se que se tratava de cisticercose, em sua forma tumoral intramedular. Após a retirada, o mesmo evoluiu de maneira satisfatória, porém sem regressões dos sintomas.

A case of spinal cord cysticercosis in a 42 year-old man is reported. He presented a six years history of paresthesia in both legs, which progressed to lack of feeling pain, spastic paraparesis of the lower limbs. On physical examination there was bilateral Babinski sign, spasticity of the lower limbs, and lack of sensitivity below T7. The magnetic resonance imaging showed an expansive lesion at the level of T5. Patient was referred for excision of the process, and while it was withdrawn, it was found that to be a cysticercosis in a shape of spinal cord tumor. There was no postoperative complication but the symptoms did not improve.

Manobra de Valsalva como preditor do resultado da cirurgia de hérnia discal lombar / Valsalva maneuver as a predictor of herniated lumbar disc surgical treatment results

Objetivo: Demonstrar o valor da resposta dolorosa à realização da manobra de Valsalva na escolhado tratamento adequado da hérnia discal lombar. Casuística e método: Estudo prospectivo de 2.200pacientes de ambos os gêneros, de diversas faixas etárias, atendidos e acompanhados no HospitalAntônio Targino e na Clínica de Neurologia e Neurocirurgia Domício Holanda, na cidade de CampinaGrande, PB, com diagnóstico de hérnia discal, no período de janeiro de 1993 a outubro de 2008. Ospacientes foram divididos em dois grupos: um grupo de 2.000 pacientes com lombociatalgia decorrentesde hérnias lombares que apresentavam dor durante a manobra de Valsalva e outro grupo de 200 pacientes que tinham as mesmas características clínicas do primeiro grupo, porém não apresentavam exacerbação da dor durante manobra de Valsalva. Resultado: Os 2.000 pacientes que apresentaram dor com a execução da manobra e foram submetidos à cirurgia tiveram boa resposta, enquanto os outros200 pacientes que não referiram a mesma dor e que foram seguidos de maneira ambulatorial tiveramuma diminuição dessa dor, porém não precisaram de tratamento cirúrgico. Conclusão: A dor provocada pela manobra de Valsalva é sinal extremamente útil no que se refere à triagem desses pacientes.

Valsalva maneuver as a predictor of herniated lumbar disc surgical treatment resultsObjective: Demonstrate the value of pain response to the Valsalva maneuver in choosing the appropriate treatment of lumbar disc herniation. Patients and methods: Prospective study of 2,200 patients of both genders in various age groups, treated and followed at Hospital Antônio Targino and Clínica de Neurologia e Neurocirurgia Domício Holanda, Campina Grande, PB, Brazil with disc herniation in theperiod from January 1993 to and in October 2008. Patients were divided into two groups: one group of2,000 patients with herniated lumbar disc with radicular pain that presented pain to maneuver Valsalvaand another group of 200 patients who had similar clinical characteristics of the first group, but showed no exacerbation of pain during maneuver of Valsalva. Results: The 2,000 patients from the first group (Valsalva-positive) underwent surgery and had a good response, while the other 200 Valsalva-negativepatients were followed in outpatient settings, had the pain decreased but did not require surgical treatment. Conclusion: The pain caused by the Valsalva maneuver is extremely useful signal with regard to triage these patients.