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1.
Childs Nerv Syst ; 40(8): 2271-2278, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38884778

RESUMEN

INTRODUCTION: Pediatric-type diffuse low-grade gliomas are a new entity that was introduced in the fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System, which was published in 2021. Notably, the information regarding the radiophenotypes of this new entity is limited. OBJECTIVE: T2-FLAIR mismatch sign has been mostly studied in adult-type diffuse gliomas so far. We aimed to present more pediatric cases for future research about T2-FLAIR mismatch signs in pediatric-type diffuse low-grade gliomas. CASE PRESENTATION: The current study presents a case of a 2-year-old boy who has a subcortical tumor at the right precentral frontal region. This tumor exhibited a T2-fluid-attenuated inversion recovery (FLAIR) mismatch sign that was identified as specific for isocitrate dehydrogenase (IDH)-mutant 1p/19q non-co-deleted astrocytomas. The tumor was pathologically identified as pediatric-type diffuse low-grade gliomas, and it tested negative for IDH-1 immunohistochemistry. The whole-exome sequencing of tumor tissue revealed negative results for IDH mutation, 1p/19q co-deletion, MYB rearrangement, and all other potential pathogenic mutations. CONCLUSION: The T2-FLAIR mismatch sign may not be 100% specific for IDH-mutant gliomas, especially in children, and researchers must further investigate the pathophysiology of the T2-FLAIR mismatch sign in brain tumors and the radiophenotypes of entities of pediatric brain tumors.


Asunto(s)
Neoplasias Encefálicas , Glioma , Humanos , Masculino , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/diagnóstico por imagen , Preescolar , Glioma/genética , Glioma/diagnóstico por imagen , Glioma/patología , Imagen por Resonancia Magnética , Isocitrato Deshidrogenasa/genética
2.
Acta Neurochir (Wien) ; 166(1): 88, 2024 Feb 19.
Artículo en Inglés | MEDLINE | ID: mdl-38372820

RESUMEN

BACKGROUND: Resuming professional activity after awake surgery for diffuse low-grade glioma (DLGG) is an important goal, which is not reached in every patient. Cognitive deficits can occur and persist after surgery. In this study, we analyzed the impact of mild cognitive impairments on the work resumption. METHODS: Fifty-four surgeries (including five redo surgeries) performed between 2012 and 2020 for grade 2 (45) and 3 (nine) DLGG in 49 professionally active patients (mean age 40 [range 23-58.) were included. We retrospectively extracted the results of semantic and phonemic verbal fluency tests from preoperative and 4-month postoperative cognitive assessments. Patients were interviewed about their working life after surgery, between April and June 2021. RESULTS: Patients (85%) returned to work, most within 3 to 6 months. Patients (76%) reported subjective complaints (primarily fatigue). Self-reported symptoms and individual and clinical variables had no impact on the work resumption. Late-postoperative average Z-scores in verbal fluency tasks were significantly lower than preoperative for the entire cohort (Wilcoxon test, p < 0.001 for semantic and p = 0.008 for phonemic fluency). The decrease in Z-scores was significantly greater (Mann Whitney U-test, semantic, p = 0.018; phonemic, p = 0.004) in the group of patients who did not return to work than in the group of patients who did. CONCLUSION: The proportion of patients returning to work was comparable to similar studies. A decrease in verbal fluency tasks could predict the inability to return to work.


Asunto(s)
Neoplasias Encefálicas , Trastornos del Conocimiento , Glioma , Humanos , Adulto , Neoplasias Encefálicas/cirugía , Estudios Retrospectivos , Vigilia , Glioma/cirugía
3.
J Magn Reson Imaging ; 58(3): 741-749, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-36524459

RESUMEN

BACKGROUND: The human brain has ability to reorganize itself in response to glioma. However, the mechanism of cortical reorganization remains unclear. PURPOSE: To investigate alterations in cortical thickness and local gyration index (LGI) in patients with unilateral frontal lobe diffuse low-grade glioma (DLGG). STUDY TYPE: Retrospective. SUBJECTS: Ninety-nine patients with histopathologically proven DLGG invading the left frontal lobe (LF; N = 56) or the right frontal lobe (RF; N = 43), and healthy controls (HC; N = 53). FIELD STRENGTH/SEQUENCE: 3.0 T, 3D T1-weighted images and gadolinium enhanced T1-weighted images using magnetization-prepared rapid gradient echo sequence, T2-weighted images, and fluid-attenuated inversion recovery using turbo spin echo sequence. ASSESSMENT: In patients with DLGG, virtual brain grafting combined with Freesurfer was utilized to enable automated cortical thickness and LGI calculation. In HC, standard FreeSurfer pipeline was applied to calculate these measures. Radiomic features were extracted from glioma using Pyradiomic software. STATISTICAL TESTS: General linear model and Pearson's correlation analysis. A P value <0.05 was considered statistically significant. RESULTS: For LF patients, there was significantly increased cortical thickness in the rostral middle frontal gyrus, significantly reduced cortical thickness in the precentral gyrus and hypogyrification in the lingual and medial orbitofrontal (MOF) gyrus in contralateral hemisphere. For RF patients, there was significantly increased cortical thickness in the middle temporal, lateral occipital extending to isthmus cingulate gyrus, significantly reduced cortical thickness in the precentral gyrus and hypogyrification in the lingual gyrus in the contralateral hemisphere. A negative association between four textural features of DLGG and LGI in the right MOF gyrus of LF group was found (r = -0.609, -0.442, -0.545, and -0.417, respectively). DATA CONCLUSION: Cortical thickness compensation was shown in contralateral homotopic location and some distant contralateral regions. Additionally, there was decreased cortical thickness in the contralateral precentral gyrus and hypogyrification in contralateral lingual gyrus. EVIDENCE LEVEL: 4 TECHNICAL EFFICACY: Stage 2.


Asunto(s)
Encéfalo , Corteza Motora , Humanos , Estudios Retrospectivos , Giro del Cíngulo , Imagen por Resonancia Magnética/métodos
4.
Acta Neurochir (Wien) ; 165(10): 2755-2767, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37672097

RESUMEN

PURPOSE: The main objective was to assess the neuropsychological, epileptical, and oncological outcomes in a series of patients operated on for a IDH-mutated diffuse low-grade glioma (DLGG) of incidental discovery (iDLGG). METHODS: We retrospectively reviewed a consecutive series of surgically treated adults with DLGG and selected cases incidentally discovered. Tumor volumes, growth rates, and extents of resection (EOR) were assessed by volumetric measures of fluid-attenuated inversion recovery magnetic resonance imaging. The data on oncological, functional, and epileptical results were retrieved from the patients' digital files. RESULTS: Among all patients with DLGG resected at our center between June 2011 and April 2022, we found eleven cases with an incidental discovery. Resection was supratotal, gross total, and subtotal in 45.5%, 26.4%, and 18.1% of cases, respectively. The rate of epileptic seizures after the surgery was 9.1%. There were 45.4% of patients that had tumor progressions and the overall mean time to tumor progression was 42 months. After the surgery, 3 (27.3%) patients had mild neurocognitive deteriorations, which impeded the return to work in one patient (9.1%). There were no differences with previous series regarding clinical, radiological, and molecular characteristics. Similar results were also found for functional, surgical, epileptical, and oncological outcomes. CONCLUSION: Although the right approach for iDLGG is still a matter of debate, our data support the safety and effectiveness of early surgical resection. More studies are needed to firmly ground this early "preventive" surgery approach.


Asunto(s)
Neoplasias Encefálicas , Glioma , Adulto , Humanos , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirugía , Estudios Retrospectivos , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Glioma/diagnóstico por imagen , Glioma/genética , Glioma/cirugía
5.
Pathologica ; 114(6): 410-421, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36534420

RESUMEN

The WHO 2021 classification of central nervous system cancers distinguishes diffuse gliomas that arise in adults (referred to as the "adult type") and those that arise in children (defined as "paediatric") based on clinical and molecular characteristics."). However, paediatric-type gliomas may occasionally be present in younger adults and occasionally adult-type gliomas may occur in children. Diffuse low-grade paediatric glioma includes diffuse astrocytoma altered by MYB or MYBL1, low-grade polymorphic juvenile neuroepithelial tumour, angiocentric glioma, and diffuse low-grade glioma with an altered MAPK pathway. Here, we examine these newly recognised entities according to WHO diagnostic criteria and propose an integrated diagnostic approach that can be used to separate these clinically and biologically distinct tumor groups.


Asunto(s)
Neoplasias Encefálicas , Glioma , Adulto , Niño , Humanos
6.
Acta Neurochir (Wien) ; 163(5): 1257-1267, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33576912

RESUMEN

BACKGROUND: Cognitive functioning is generally well preserved in patients with diffuse low-grade glioma (DLGG), even in the case of extended tumor and resection. To date, the question of personality changes in these patients has received little attention. Our aim was to investigate to what extent certain aspects of personality and behaviors could be affected by DLGG resection. METHODS: We used self-reported personality questionnaires (NOEPI-R and TCI-R) and hetero-evaluation of executive behavioral changes in a large sample of 98 patients operated on for DLGG. To compare the patients' scores from the personality questionnaires, we recruited 47 healthy controls participants. To identify the putative neural networks associated with behavioral changes, a combination of voxel-wise and tract-wise lesion-symptom mapping was performed. RESULTS: First, results revealed no difference between patients and controls for each subdimension of the NOEPI-R. Regarding the TCI-R, the character dimensions and three out of four temperament dimensions did not differ. Second, behavioral changes (Irritability, Hypoactivity, Anticipative disorders, and disinterest) were reported between 40 and 50% of cases. Third, some personality dimensions (as neuroticism) were strongly predictive of postoperative behavioral disorders (as hypoactivity). Lastly, specific behavioral changes were associated with selective damage to cortical (left inferior frontal gyrus, supplementary motor area, and right fusiform gyrus) and white matter (left inferior fronto-occipital and uncinate fasciculi, right cingulum) structures. CONCLUSION: This study demonstrates that extensive lesions caused by DLGGs and their surgical resection have no or minor impact on patients' personality. However, specific personality dimensions are strongly predictive of behavioral disorders suggesting that the observed surgically related behavioral changes are modulated by the personality profile. Finally, the lesion mapping analyses indicate that damage to differential cortical or white matter structures leads to distinct patterns of behavioral abnormalities.


Asunto(s)
Conducta , Mapeo Encefálico , Neoplasias Encefálicas/cirugía , Personalidad , Adolescente , Adulto , Anciano , Neoplasias Encefálicas/patología , Estudios de Casos y Controles , Femenino , Glioma/patología , Glioma/cirugía , Humanos , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Encuestas y Cuestionarios , Sustancia Blanca/patología , Sustancia Blanca/cirugía , Adulto Joven
7.
J Neurooncol ; 146(2): 329-337, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31883050

RESUMEN

BACKGROUND: Despite aspirations to achieve equality in healthcare we know that socioeconomic differences exist and may affect treatment and patient outcome, also in serious diseases such as cancer. We investigated disparities in neurosurgical care and outcome for patients with low-grade glioma (LGG). METHODS: In this nationwide registry-based study, patients who had undergone surgery for LGG during 2005-2015 were identified (n = 547) through the Swedish Brain Tumor Registry. We linked data to multiple national registries with individual level data on income, education and comorbidity and analyzed the association of disease characteristics, surgical management and outcome, with levels of income, education and sex. RESULTS: Patients with either low income, low education or female gender showed worse pre-operative performance status. Patients with low income or education also had more comorbidities and those with low education endured longer waiting times for surgery. Median time from radiological imaging to surgery was 51 days (Q1-3 27-191) for patients with low education, compared to 32 days (Q1-3 20-80) for patients with high education (p = 0.006). Differences in waiting time over educational levels remained significant after stratification for age, comorbidity, preoperative performance status, and tumor size. Overall survival was better for patients with high income or high education, but income- and education-related survival differences were not significant after adjustment for age and comorbidity. The type of surgical procedure or complications did not differ over socioeconomic groups or sex. CONCLUSION: The neurosurgical care for LGG in Sweden, a society with universal healthcare, displays differences that can be related to socioeconomic factors.


Asunto(s)
Neoplasias Encefálicas/terapia , Atención a la Salud/estadística & datos numéricos , Glioma/terapia , Renta/estadística & datos numéricos , Sistema de Registros/estadística & datos numéricos , Adulto , Neoplasias Encefálicas/economía , Neoplasias Encefálicas/patología , Comorbilidad , Femenino , Estudios de Seguimiento , Glioma/economía , Glioma/patología , Humanos , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Factores Socioeconómicos , Tasa de Supervivencia , Suecia
8.
Cogn Neuropsychol ; 36(3-4): 140-157, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-29879863

RESUMEN

The role of the left inferior longitudinal fasciculus (ILF) in language processing has been called into question by recent studies showing that disruption of this tract in glioma patients did not necessarily lead to detrimental effects on spoken language, especially on picture naming. Here we show that disruption of the left ILF with axonal stimulation in patients undergoing an "awake" surgery for a slow-growing tumour systematically induces pure anomia, but only when the temporal pole (TP) is not infiltrated by the tumour. This finding not only confirms that the ILF plays a role in lexical retrieval in normal circumstances but also suggests that the information conveyed by this tract can be rerouted to alternative pathways when the TP is widely lesioned and abandons its function. This conclusion is further supported by the case of a patient who developed a long-lasting anomic aphasia after a surgically preplanned interruption of the ILF.


Asunto(s)
Mapeo Encefálico/métodos , Adulto , Corteza Cerebral/fisiopatología , Femenino , Humanos , Lenguaje , Masculino , Persona de Mediana Edad
9.
Glia ; 66(2): 239-255, 2018 02.
Artículo en Inglés | MEDLINE | ID: mdl-29027701

RESUMEN

Diffuse low grade gliomas (DLGG, grade II gliomas) are slowly-growing brain tumors that often progress into high grade gliomas. Most tumors have a missense mutation for IDH1 combined with 1p19q codeletion in oligodendrogliomas or ATRX/TP53 mutations in astrocytomas. The phenotype of tumoral cells, their environment and the pathways activated in these tumors are still ill-defined and are mainly based on genomics and transcriptomics analysis. Here we used freshly-resected tumors to accurately characterize the tumoral cell population and their environment. In oligodendrogliomas, cells express the transcription factors MYT1, Nkx2.2, Olig1, Olig2, Sox8, four receptors (EGFR, PDGFRα, LIFR, PTPRZ1) but not the co-receptor NG2 known to be expressed by oligodendrocyte progenitor cells. A variable fraction of cells also express the more mature oligodendrocytic markers NOGO-A and MAG. DLGG cells are also stained for the young-neuron marker doublecortin (Dcx) which is also observed in oligodendrocytic cells in nontumoral human brain. In astrocytomas, MYT1, PDGFRα, PTPRZ1 were less expressed whereas Sox9 was prominent over Sox8. The phenotype of DLGG cells is overall maintained in culture. Phospho-array screening showed the absence of EGFR and PDGFRα phosphorylation in DLGG but revealed the strong activation of p44/42 MAPK/ERK which was present in a fraction of tumoral cells but also in nontumoral cells. These results provide evidence for the existence of close relationships between the cellular phenotype and the mutations found in DLGG. The slow proliferation of these tumors may be associated with the absence of activation of PDGFRα/EGFR receptors.


Asunto(s)
Neoplasias Encefálicas/genética , Receptores ErbB/genética , Glioma/genética , Isocitrato Deshidrogenasa/genética , Mutación/genética , Receptor alfa de Factor de Crecimiento Derivado de Plaquetas/genética , Adulto , Animales , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/metabolismo , Neoplasias Encefálicas/metabolismo , Neoplasias Encefálicas/patología , Proteína Doblecortina , Receptores ErbB/metabolismo , Femenino , Glioma/metabolismo , Glioma/patología , Proteína Homeobox Nkx-2.2 , Proteínas de Homeodominio , Humanos , Isocitrato Deshidrogenasa/metabolismo , Masculino , Ratones , Persona de Mediana Edad , Clasificación del Tumor/métodos , Proteínas Nucleares , Receptor alfa de Factor de Crecimiento Derivado de Plaquetas/metabolismo , Factores de Transcripción , Células Tumorales Cultivadas , Adulto Joven
10.
Brain Cogn ; 120: 48-57, 2018 02.
Artículo en Inglés | MEDLINE | ID: mdl-29122369

RESUMEN

Brodmann area 10 (BA10) is thought to be at the summit of the prefrontal cortex's hierarchical organization. It is widely accepted that metacognitive abilities depend on the structural and functional properties of BA10. Our objective was to assess whether metacognition can be maintained after low-grade glioma surgery with BA10 resection. Three groups of participants were recruited: (i) patients having undergone resection of the right prefrontal cortex, including BA10 (n = 9); (ii) patients having undergone resection of the right prefrontal cortex but not BA10 (n = 10); and (iii) healthy controls (n = 38). Importantly, we also included a patient (referred to as "PR") with resection of BA10 in the two hemispheres. The patients with resection of right BA10 had metacognitive performances that were indistinguishable from those of brain-damaged control patients and healthy controls. Crucially, PR's metacognitive ability was not only maintained but was even in the upper quartile of normal performances. Our findings demonstrate that the brain can redistribute and remap metacognition in response to injury. We thus provide experimental evidence against the conventional hypothesis whereby cognitive functions are directly and lastingly linked to particular cortical structures. The latter hypothesis seems to be particularly false for the highest levels of human cognition and for BA10.


Asunto(s)
Neoplasias Encefálicas/cirugía , Metacognición/fisiología , Plasticidad Neuronal/fisiología , Corteza Prefrontal/patología , Corteza Prefrontal/fisiología , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Corteza Prefrontal/cirugía
11.
Brain Cogn ; 125: 45-52, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29870873

RESUMEN

Slow-growing, infiltrative brain tumours may modify the electrophysiological balance between the two hemispheres. To determine whether and how asymmetry of EEG rhythms during motor preparation might occur following "awake brain surgery" for this type of tumour, we recorded electroencephalograms during a simple visuo-manual reaction time paradigm performed by the patients between 3 and 12 months after surgery and compared them to a control group of 8 healthy subjects. Frequency analyses revealed imbalances between the injured and healthy hemispheres. More particularly, we observed a power increase in the δ frequency band near the lesion site and a power increase in the α and ß frequency bands. Interestingly, these alterations seem to decrease for the two patients whose surgery were anterior to 9 months, independently of the size of the lesion. Reaction times did not reflect this pattern as they were clearly not inversely related to the anteriority of the surgery. Electrophysiology suggests here different processes of recovery compared to behavioral data and brings further insights for the understanding of EEG rhythms that should not be systematically confounded or assimilated with cognitive performances. EEG monitoring is rare for these patients, especially after awake brain surgery, however it is important.


Asunto(s)
Neoplasias Encefálicas/fisiopatología , Encéfalo/fisiopatología , Cognición/fisiología , Tiempo de Reacción/fisiología , Vigilia/fisiología , Adulto , Neoplasias Encefálicas/cirugía , Electroencefalografía , Femenino , Humanos , Masculino , Persona de Mediana Edad
12.
Int J Neurosci ; 128(9): 886-890, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-29384395

RESUMEN

Diffuse Low-Grade Gliomas (LGGs) include World Health Organization (WHO) grade II diffuse astrocytoma, oligodendroglioma and oligoastrocytoma. Since the neurological symptoms of LGGs are often subtle and deceptive, LGGs are easily overlooked at their early stage. Here, we report a case of a 49-year-old female with symptoms and imaging manifestations mimicking ischaemic infarct. After treatments for ischaemic stroke, the symptoms initially fluctuated and then aggravated. In addition, we found that the locations of the lesions did not match the vascular distribution and no obvious abnormalities were observed by Computed Tomography (CT) angiography and transcranial Doppler. The results from the Magnetic Resonance Spectroscopy (MRS) and from the stereotactic biopsy directed to the final diagnosis of WHO grade II, isocitrate dehydrogenase (IDH)-wild-type diffuse astrocytoma. This is the first reported LGG case with a stroke-like onset. This case illustrates how easy it is to misdiagnose an LGG as a stroke if just using cerebral CT and magnetic resonance imaging. MRS and biopsy can assist with the differential diagnosis process thereby avoiding inappropriate or delayed treatments.


Asunto(s)
Isquemia Encefálica/fisiopatología , Neoplasias Encefálicas/fisiopatología , Glioma/diagnóstico por imagen , Glioma/fisiopatología , Isquemia Encefálica/diagnóstico por imagen , Neoplasias Encefálicas/diagnóstico por imagen , Femenino , Glioma/metabolismo , Humanos , Imagen por Resonancia Magnética , Espectroscopía de Resonancia Magnética , Persona de Mediana Edad , Proteínas del Tejido Nervioso/metabolismo , Tomografía Computarizada por Rayos X
13.
J Neurooncol ; 133(1): 137-146, 2017 05.
Artículo en Inglés | MEDLINE | ID: mdl-28401374

RESUMEN

Early resection is standard of care for presumed low-grade gliomas. This is based on studies including only tumors that were post-surgically confirmed as low-grade glioma. Unfortunately this does not represent the clinicians' situation wherein he/she has to deal with a lesion on MRI that is suspect for low-grade glioma (i.e. without prior knowledge on the histological diagnosis). We therefore aimed to determine the optimal initial strategy for patients with a lesion suspect for low-grade glioma, but not histologically proven yet. We retrospectively identified 150 patients with a resectable presumed low-grade-glioma and who were otherwise in good clinical condition. In this cohort we compared overall survival between three types of initital treatment strategy: a wait-and-scan approach (n = 38), early resection (n = 83), or biopsy for histopathological verification (n = 29). In multivariate analysis, no difference was observed in overall survival for early resection compared to wait-and-scan: hazard ratio of 0.92 (95% CI 0.43-2.01; p = 0.85). However, biopsy strategy showed a shorter overall survival compared to wait-and-scan: hazard ratio of 2.69 (95% CI 1.19-6.06; p = 0.02). In this cohort we failed to confirm superiority of early resection over a wait-and-scan approach in terms of overall survival, though longer follow-up is required for final conclusion. Biopsy was associated with shorter overall survival.


Asunto(s)
Neoplasias Encefálicas/cirugía , Glioma/cirugía , Adulto , Biopsia , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/patología , Tratamiento Conservador , Femenino , Estudios de Seguimiento , Glioma/diagnóstico por imagen , Glioma/patología , Humanos , Estimación de Kaplan-Meier , Masculino , Análisis Multivariante , Clasificación del Tumor , Modelos de Riesgos Proporcionales , Estudios Retrospectivos
14.
Acta Neurochir (Wien) ; 159(7): 1167-1178, 2017 07.
Artículo en Inglés | MEDLINE | ID: mdl-28474122

RESUMEN

BACKGROUND: The European Low-Grade Glioma network indicated a need to better understand common practices regarding the managing of diffuse low-grade gliomas. This area has experienced great advances in recent years. METHOD: A general survey on the managing of diffuse low-grade gliomas was answered by 21 centres in 11 European countries. Here we focused on specific questions regarding perioperative and intraoperative cognitive assessments. RESULTS: More centres referred to the same speech and language therapist and/or neuropsychologist across all assessments; a core of assessment tools was routinely used across centres; fluency tasks were commonly used in the perioperative stages, and object naming during surgery; tasks that tapped on attention, executive functions, visuospatial awareness, calculation and emotions were sparsely administered; preoperative assessments were performed 1 month or 1 week before surgery; timing for postoperative assessments varied; finally, more centres recommended early rehabilitation, whenever needed. CONCLUSIONS: There is an emerging trend towards following similar practices for the management of low-grade gliomas in Europe. Our results are descriptive and formalise current discussions in our group. Also, they contribute towards the development of a European assessment protocol.


Asunto(s)
Neoplasias Encefálicas/cirugía , Cognición , Glioma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Complicaciones Posoperatorias/prevención & control , Guías de Práctica Clínica como Asunto , Neoplasias Encefálicas/diagnóstico , Europa (Continente) , Glioma/diagnóstico , Humanos , Procedimientos Neuroquirúrgicos/efectos adversos , Procedimientos Neuroquirúrgicos/normas , Periodo Preoperatorio
15.
J Neurooncol ; 130(2): 289-298, 2016 11.
Artículo en Inglés | MEDLINE | ID: mdl-27161250

RESUMEN

Once considered a "no man's land" especially when invaded by a diffuse low grade glioma (DLGG), the insula remains to this day a surgical challenge. Surgery for insular DLGG involves consideration of its hidden location under the potentially eloquent operculae, the proximity to vascular tree and high density of functions not only in the insular cortex but also in the white fiber pathways passing under the insular lobe. The natural history of DLGG and the potential benefits and consequences of the surgical approach also need a close look. In the last decade, a better knowledge of the functional anatomy and connectivity of this region, as well as an improvement in surgical techniques as direct stimulation mapping, combined with an increasing literature showing a favorable impact of maximal resection for DLGG, were deciding factors in the paradigmatic shift from expectative treatment to early surgical management. Here, our goal is to discuss the structural and functional aspects of the insula, the specificities of insular and paralimbic DLGG by emphasizing the technical considerations of surgery in this region, as well as its oncological and functional outcomes. In summary, this new strategy based upon early maximal safe surgical resection showed both oncological benefit and preservation of quality of life-or even an improvement thanks to epilepsy relief.


Asunto(s)
Neoplasias Encefálicas/cirugía , Corteza Cerebral/cirugía , Glioma/cirugía , Procedimientos Neuroquirúrgicos , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/patología , Corteza Cerebral/diagnóstico por imagen , Corteza Cerebral/patología , Glioma/diagnóstico por imagen , Glioma/patología , Humanos , Imagen por Resonancia Magnética , Clasificación del Tumor , Resultado del Tratamiento
16.
Neurosurg Rev ; 39(3): 377-84, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26610909

RESUMEN

Although a large amount of data supports early surgical resection for symptomatic diffuse low-grade glioma, the therapeutic strategy is still a matter of debate regarding incidentally discovered diffuse low-grade glioma. Indeed, early and "preventive" surgery has recently been proposed in asymptomatic patients with silent diffuse low-grade glioma with better outcomes. The present review discusses the importance of an early diagnosis and of a preventive surgical treatment to improve the outcomes of incidental diffuse low-grade glioma and suggests the possible relevance of a tailored screening policy.


Asunto(s)
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirugía , Diagnóstico Precoz , Glioma/diagnóstico , Glioma/cirugía , Humanos , Clasificación del Tumor , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento
17.
Acta Neurochir (Wien) ; 158(1): 51-8, 2016 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-26530708

RESUMEN

BACKGROUND: Total or subtotal surgical resection of WHO grade II glioma (diffuse low-grade glioma, DLGG) can significantly increase survival. Moreover, a supratotal resection, i.e., an extended resection with a margin beyond MR imaging abnormalities, could decrease the risk of malignant transformation. Here, the goal is to analyze the long-term functional and oncological outcomes following supratotal resection for DLGG. METHODS: Sixteen consecutive patients who underwent supratotal resection for a DLGG with a minimum follow-up of 8 years after surgery were included. The resection was continued up to functional cortical and subcortical structures defined by intrasurgical electrical mapping. The extent of resection was evaluated on postoperative FLAIR-weighted MR imaging. Data regarding clinicoradiological features, therapeutic management, and outcomes were analyzed. RESULTS: Seven men and nine women (mean age, 41.3 years, range, 26-63 years) were included (seizure in 15 cases, one incidental discovery). All patients resumed a normal life after surgery (no neurological deficits, no epilepsy). The volume of postoperative cavity was larger than the preoperative tumor volume in the 16 patients. Neuropathological examination confirmed the diagnosis of WHO grade II glioma in all cases. No adjuvant treatment was administrated after resection. The mean duration of postoperative follow-up was 132 months (range, 97-198 months). There was no relapse in eight cases. Eight patients experienced tumor recurrence, with an average time to relapse of 70.3 months (range, 32-105 months), but without malignant transformation. Five of them have been re-treated, with a reoperation (two cases), chemotherapy (three cases) and radiotherapy (two cases). All patients continue to enjoy a normal life. CONCLUSIONS: This is the first series demonstrating the prolonged impact of supratotal resection on malignant transformation of DLGG. These original data may suggest to remove a margin around the FLAIR-weighted MR imaging abnormalities in a more systematic manner for DLGG not involving eloquent structures.


Asunto(s)
Neoplasias Encefálicas/cirugía , Glioma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Adulto , Neoplasias Encefálicas/patología , Femenino , Estudios de Seguimiento , Glioma/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Recurrencia Local de Neoplasia/epidemiología , Enfermedades del Sistema Nervioso/epidemiología , Complicaciones Posoperatorias/epidemiología , Estudios Prospectivos , Recuperación de la Función , Reoperación/estadística & datos numéricos , Convulsiones/etiología , Convulsiones/cirugía , Resultado del Tratamiento
18.
Neuro Oncol ; 26(7): 1327-1334, 2024 Jul 05.
Artículo en Inglés | MEDLINE | ID: mdl-38466086

RESUMEN

BACKGROUND: Pediatric-type diffuse low-grade gliomas (pLGG) harboring recurrent genetic alterations involving MYB or MYBL1 are closely related tumors. Detailed treatment and outcome data of large cohorts are still limited. This study aimed to comprehensively evaluate pLGG with these alterations to define optimal therapeutic strategies. METHODS: We retrospectively reviewed details of pLGG with MYB or MYBL1 alterations from patients treated or referred for pathologic review at St. Jude Children's Research Hospital. Tumor specimens were centrally reviewed, and clinical data were collated. RESULTS: Thirty-three patients (18 male; median age, 5 years) were identified. Two tumors had MYBL1 alterations; 31 had MYB alterations, MYB::QKI fusion being the most common (n = 10, 30%). Most tumors were in the cerebral hemispheres (n = 22, 67%). Two patients (6%) had metastasis at diagnosis. The median follow-up was 6.1 years. The 5-year event-free survival (EFS) rate was 81.3% ±â€…8.3%; the 5-year overall survival (OS) rate was 96.4% ±â€…4.1%. Patients receiving a near-total or gross-total resection had a 5-year EFS of 100%; those receiving a biopsy or subtotal resection had a 5-year EFS rate of 56.6% ±â€…15.2% (P < .01). No difference in EFS was observed based on location, histology, or molecular alterations. However, the tumors that progressed or metastasized may have distinct methylation profiles with evidence of activation of the MAPK and PI3K/AKT/mTOR pathways. CONCLUSIONS: pLGG with MYB/MYBL1 alterations have good outcomes. Our findings suggest that surgical resectability is a crucial determinant of EFS. Further characterization is required to identify optimal treatment strategies for progressive tumors.


Asunto(s)
Neoplasias Encefálicas , Glioma , Proteínas Proto-Oncogénicas c-myb , Niño , Preescolar , Femenino , Humanos , Masculino , Biomarcadores de Tumor/genética , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/genética , Estudios de Seguimiento , Glioma/patología , Glioma/genética , Clasificación del Tumor , Pronóstico , Proteínas Proto-Oncogénicas , Proteínas Proto-Oncogénicas c-myb/genética , Estudios Retrospectivos , Tasa de Supervivencia , Transactivadores/genética
19.
CNS Neurosci Ther ; 29(2): 736-743, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36514187

RESUMEN

AIMS: We aimed to identify predictors of postoperative seizures in patients with diffuse low-grade glioma (DLGG)-related epilepsy after complete tumor resection in this study. METHODS: We retrospectively collected data from individuals with DLGG-related epilepsy whose tumors were completely resected at Xiangya Hospital, Central South University between January 2014 and January 2020. The predictors of seizure outcomes were assessed by employing univariate analysis and a multivariate logistic regression model in a backward binary logistic regression model. RESULTS: Among the 118 cases that met the inclusion criteria, 83.05% were seizure-free following an average follow-up of 4.27 ± 1.65 years, all of whom were classified as International League Against Epilepsy class I outcome. Univariate and multivariate analyses indicated that seizure duration of >6 years (odds ratio [OR], 6.62; 95% confidence interval [CI], 1.76-24.98; p = 0.005) and first clinical symptoms other than seizures (OR, 4.51; 95% CI, 1.43-14.23; p = 1.010) were both independent predictors of unfavorable seizure outcomes. CONCLUSION: Our results imply that satisfactory seizure outcomes can be achieved in most patients with DLGG-related epilepsy after complete tumor resection. Patients with seizure duration of >6 years or first clinical symptoms other than seizures were more likely to experience postoperative seizure recurrence.


Asunto(s)
Neoplasias Encefálicas , Epilepsia , Glioma , Humanos , Estudios Retrospectivos , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Resultado del Tratamiento , Glioma/complicaciones , Glioma/cirugía , Glioma/patología , Convulsiones/epidemiología , Convulsiones/etiología , Convulsiones/cirugía , Epilepsia/etiología , Epilepsia/cirugía , Epilepsia/patología
20.
Anticancer Res ; 43(6): 2659-2670, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37247932

RESUMEN

BACKGROUND/AIM: In the latest 2021 WHO classification of central nervous system tumours (CNS), gliomas that present isocitrate dehydrogenase (IDH) mutations are defined as diffuse low-grade gliomas (DLGGs). IDH mutations are commonly observed in this tumour type. The Extent of Resection (EOR) positively influence survival; however, it is still debated whether the predictive value of EOR is independent of the 1p/19q co-deletion. We carried out a retrospective analysis on patients operated on for DLGG at the Sant'Andrea University Hospital Sapienza University of Rome, correlating the outcome with the presence of 1p/19q co-deletion and EOR. PATIENTS AND METHODS: The study examined 66 patients with DLGG who had undergone surgery for tumour resection between 2008 and 2018. Patients with DLGG were divided into two groups; diffuse astrocytoma (DA) in which 1p/19q codeletion is absent and oligodendroglioma (OG) in which 1p/19q codeletion is present. According to EOR, both groups were divided into two subgroups: subtotal resection (STR) and gross total resection (GTR). Three end-point variables were considered: overall survival (OS), progression-free survival (PFS) and time to malignant transformation (TMT). RESULTS: In the DA group, the GTR subgroup had an average OS of 81.6 months, an average PFS of 45.9 months and an average TMT of 63.6 months. After surgery, these patients had an average Karnofsky Performance Score (KPS) of 83.4. The STR subgroup had an average OS of 60.4 months, PFS was 38.7 months, and TMT was 46.4 months, post-operative KPS was 83.4. In contrast, in the OG group, the GTR averagely had 101.7 months of OS, 64.9 months of PFS, 80.3 months of TMT and an average post-operative KPS of 84.2, and the STR subgroup had an average of OS of 73.3 months, PFS of 48.2 months, TMT of 57.3 and an average postoperative KPS of 96.2. CONCLUSION: In patients affected by DLGGs, 1p/19q codeletion is significantly associated with prolonged survival and longer time-to-malignant transformation (TMT) compared to the absence of 1p/19q codeletion. Also, the extent of surgical resection (EOR) in DLGG patients has been confirmed as one of the main prognostic factors. However, its predictive value is substantially influenced by the presence of the 1p/19q codeletion.


Asunto(s)
Astrocitoma , Neoplasias Encefálicas , Glioma , Oligodendroglioma , Humanos , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Estudios Retrospectivos , Glioma/genética , Glioma/cirugía , Glioma/patología , Aberraciones Cromosómicas , Pronóstico , Mutación , Isocitrato Deshidrogenasa/genética , Cromosomas Humanos Par 1/genética , Cromosomas Humanos Par 19/genética
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