Gliomatosis cerebri: no evidence for a separate brain tumor entity

Abstract Gliomatosis cerebri (GC) is presently considered a distinct astrocytic glioma entity according to the WHO classification for CNS tumors. It is characterized by widespread, typically bilateral infiltration of the brain involving three or more lobes. Genetic studies of GC have to date been restricted to the analysis of individual glioma-associated genes, which revealed mutations in the isocitrate dehydrogenase 1 (IDH1) and tumor protein p53 (TP53) genes in subsets of patients. Here, we report on a genome-wide analysis of DNA methylation and copy number aberrations in 25 GC patients. Results were compared with those obtained for 105 patients with various types of conventional, i.e., non-GC gliomas including diffuse astrocytic gliomas, oligodendrogliomas and glioblastomas. In addition, we assessed the prognostic role of methylation profiles and recurrent DNA copy number aberrations in GC patients. Our data reveal that the methylation profiles in 23 of the 25 GC tumors corresponded to either IDH mutant astrocytoma (n = 6), IDH mutant and 1p/19q codeleted oligodendroglioma (n = 5), or IDH wild-type glioblastoma including various molecular subgroups, i.e., H3F3A-G34 mutant (n = 1), receptor tyrosine kinase 1 (RTK1, n = 4), receptor tyrosine kinase 2 (classic) (RTK2, n = 2) or mesenchymal (n = 5) glioblastoma groups. Two tumors showed methylation profiles of normal brain tissue due to low tumor cell content...
Source: Acta Neuropathologica - Category: Neurology Source Type: research

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CCR Grand Rounds Dr. Jabado ’ s research focuses on elucidating genetic signatures of pediatric astrocytomas and examining how they compare to adults. These are deadly brain tumors that originate in the brain and include glioblastomas (GBM, the highest grade of astrocytomas), which are one of the deadliest cancers in humans. Her group uncovered that pediatric high-grade astrocytomas (HGA) are molecularly and genetically distinct from adult tumors. They also identified a new molecular mechanism driving pediatric HGA, namely recurrent somatic driver mutations in the tail of histone 3 variants (H3.3 and H3.1). These mut...
Source: Videocast - All Events - Category: General Medicine Tags: Upcoming Events Source Type: video
Purpose of review The current review summarizes recent advances on three important issues in neurofibromatosis type 1 (NF1) management: the identification of specific NF1 gene mutations predicting the risk for developing neurological malignancies; the molecular features of NF1-associated tumors and their differences from sporadic neoplasms; genetic, epigenetic, or microenviromental factors leading benign tumors to a malignant transformation in NF1. Recent findings The association between the risk of developing optic pathway glioma and specific germiline NF1 mutations is still debated and further studies are needed wit...
Source: Current Opinion in Oncology - Category: Cancer & Oncology Tags: BRAIN AND NERVOUS SYSTEM: Edited by Marc Sanson Source Type: research
ConclusionsA subgroup of recurrent MMRd HGG patients might benefit from Pem, especially those with anaplastic gliomas. There was a trend for a longer PFS and OS in PTS with aMMRd. Analyses for identifying additional molecular predictive factors is ongoing.Legal entity responsible for the studyThe authors.FundingHas not received any funding.DisclosureAll authors have declared no conflicts of interest.
Source: Annals of Oncology - Category: Cancer & Oncology Source Type: research
Conclusion. Based on a large series of our patients with a great variety of histological tumor types, it was concluded, that a neurosurgeon can predict fluorescence sensitivity before surgery.
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Authors: Steponaitis G, Kazlauskas A, Vaitkienė P, Deltuva VP, Mikuciunas M, Skiriutė D Abstract Background: Gliomas are the most common and aggressive among primary malignant brain tumours with significant inter- and intratumour heterogeneity in histology, molecular profile, and patient outcome. However, molecular targets that could provide reliable diagnostic and prognostic information on this type of cancer are currently unknown. Recent studies show that certain phenotypes of gliomas such as malignancy, resistance to therapy, and relapses are associated with the epigenetic alterations of tumour-specific genes....
Source: Journal of Oncology - Category: Cancer & Oncology Tags: J Oncol Source Type: research
AbstractObjective5-Aminolevulinic acid (5-ALA) –guided resection of gliomas in adults enables better delineation between tumor and normal brain, allowing improved resection and improved patients’ outcome. Recently, several reports were published regarding 5-ALA for resection of pediatric brain tumors. The aim of the study was to determine th e intracellular fluorescence of protoporphyrin IX (PPIX) in pediatric brain tumors by hyperspectral imaging and to compare it with visually observed intraoperative fluorescence.Methods5-ALA was administered orally 4  h prior to surgery. During tumor resection, the surg...
Source: Acta Neurochirurgica - Category: Neurosurgery Source Type: research
This study analyzed the genetic and other features ofIDH-wt LGGs to develop a subclassification that can be used to predict their prognosis. Clinical, histopathological, and genetic features of 35 cases of diffuseIDH-wt astrocytoma andIDH-wt anaplastic astrocytoma were analyzed. The following genetic factors were examined: mutations ofB-rapidly accelerated fibrosarcoma,telomerase reverse transcriptase promoter (TERTp),histone 3 family 3A, andalpha-thalassemia/mental retardation syndrome,X-linked; and copy number aberrations. In the univariate analysis, the following factors were associated with poor overall survival (OS): ...
Source: Brain Tumor Pathology - Category: Neurology Source Type: research
In this study, we aimed to describe the clinical characteristic and outcomes of tumor-associated SE (TASE) in a population of adult patients with glioma prospectively collected between 2013 and 2019. In the aforementioned period, we observed 26 TASE (median age: 68 years). Overall, 22 patients (85%) presented a HGG (one anaplastic astrocytoma and 21 a glioblastoma) while 4 had a LGG (two diffuse astrocytoma and two ganglioglioma). All the lesions were supratentorial, and the temporal lobe was the most frequently involved (20 patients). Fourteen patients (54%) had the SE episode as the first manifestation of the tumor; in...
Source: Epilepsy and Behavior - Category: Neurology Source Type: research
In this study, we aimed to describe the clinical characteristic and outcomes of tumor-associated SE (TASE) in a population of adult patients with glioma prospectively collected between 2013 and 2019. In the aforementioned period, we observed 26 TASE (median age: 68 years). Overall, 22 patients (85%) presented a HGG (one anaplastic astrocytoma and 21 a glioblastoma) while 4 had a LGG (two diffuse astrocytoma and two ganglioglioma). All the lesions were supratentorial, and the temporal lobe was the most frequently involved (20 patients). Fourteen patients (54%) had the SE episode as the first manifestation of the tumor; in...
Source: Epilepsy and Behaviour - Category: Neurology Authors: Tags: Epilepsy Behav Source Type: research
We present three cases of NF1 patients referred for 18F-fluorocholine PET/CT because of suspected glioma in the setting of ongoing FUMEGA (Functional and Metabolic Glioma Analysis) trial. One case turned out to be a WHO grade I ganglioglioma; the second was a high grade glioma; and the last one (negative in PET) a probable low-grade glioma.
Source: Clinical Nuclear Medicine - Category: Nuclear Medicine Tags: Interesting Images Source Type: research
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