Gliomatosis cerebri in children shares molecular characteristics with other pediatric gliomas

Abstract Gliomatosis cerebri (GC), a rare and deadly CNS neoplasm characterized by involvement of at least three cerebral lobes, predominantly affects adults. While a few small series have reported its occurrence in children, little is known about the molecular characteristics of pediatric GC. We reviewed clinical, radiological, and histological features of pediatric patients with primary GC treated at our institution over 15 years. Targeted sequencing of mutational hotspots in H3F3A, IDH1/2, and BRAF, and genome-wide analysis of DNA methylation and copy number abnormalities was performed in available tumors. Thirty-two patients [23 (72 %) with type 1 and 9 (28 %) with type 2 GC] were identified. Median age at diagnosis was 10.2 years (range 1.5–19.1). A median of 4 cerebral lobes (range 3–8) was affected at diagnosis. In addition, symmetrical bithalamic involvement was observed in 9 (28 %) patients. Twenty-two patients (69 %) had an anaplastic astrocytoma. Despite aggressive therapy, only two patients younger than 3 years at diagnosis are long-term survivors. Clustering analysis of methylation array data from 18 cases classified tumors as IDH (n = 3, 17 %), G34 (n = 4, 22 %), mesenchymal (n = 3, 17 %), and RTK I ‘PDGFRA’ (n = 8, 44 %). No tumors were classified as K27 subgroup. PDGFRA was the most commonly amplified oncogene in 4 of 22 tumors (18&nb...
Source: Acta Neuropathologica - Category: Neurology Source Type: research

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ConclusionThe strongest determinant of the patients ’ course after initial watchful waiting was the molecular tumor status. Extensive resection may increase time to progression and malignant transformation. Observation may be justified in selected patients.
Source: Journal of Neuro-Oncology - Category: Cancer & Oncology Source Type: research
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
In conclusion, CK2 appears to play a role not only in malignant glioma cells but it can also sustain the viability and proliferation of benign astrocytoma cells. The obtained antitumor effects of CK2 inhibitors significantly exceeded their mild or no effect on normal astrocytes in control, which supports the therapeutic potential of these compounds against gliomas. PMID: 31588710 [PubMed - in process]
Source: Folia Neuropathologica - Category: Neurology Tags: Folia Neuropathol Source Type: research
Conclusion: Mesial temporal LGGs are not alone and a high proportion of temporal neocortex appeared normal on preoperative MRI, may contain dual pathology. Thus, anterior temporal resection should be performed to have satisfactory seizure and oncological outcomes. PMID: 31583167 [PubMed]
Source: Surgical Neurology International - Category: Neurosurgery Tags: Surg Neurol Int Source Type: research
Conclusion: Although cases of concurrent glioma and MS remain rare, we present a case illustrating this phenomenon and explore a number of theories behind a potential causal relationship. PMID: 31583163 [PubMed]
Source: Surgical Neurology International - Category: Neurosurgery Tags: Surg Neurol Int Source Type: research
This study aims to describe characteristics and patterns of care of elderly patients with IDHm HGG included in the French POLA network dedicated to HGG.MethodsThe characteristics and patterns of care of elderly ( ≥70 years) patients IDHm HGG were compared to those of younger patients (
Source: Annals of Oncology - Category: Cancer & Oncology Source Type: research
ConclusionsBAY is well tolerated as anticipated due to specificity for mIDH1. BAY inhibited mIDH1-R132X activity as evidenced by a PD effect in pts with measurable 2-HG, with objective response in 3 pts (all gliomas).Clinical trial identificationNCT02746081.Editorial acknowledgementMedical writing assistance was provided by Laura Valenzo, PhD, of Complete Health Vizion and funded by Bayer AG.Legal entity responsible for the studyBayer AG.FundingBayer AG.DisclosureW. Wick: Non-remunerated activity/ies, patent: IDH vaccine; Non-remunerated activity/ies, patent: IDH diagnostic; Non-remunerated activity/ies, patent: APG diagno...
Source: Annals of Oncology - Category: Cancer & Oncology Source Type: research
AbstractBackgroundIn the phase III RTOG 9802 trial, median overall survival (OS) increased from 7.8 years with radiotherapy alone to 13.3 years (P = .002) with adjuvant procarbazine, lomustine, and vincristine (PCV) following surgery for low grade glioma. Although the PCV regimen improved survival, there were significant toxicities. We hypothesised more frequent toxicities would be observed in clinical practice. We aimed to identify and quantify toxicities in the real world setting in a National Neuro-Oncology centre.MethodsPatients with low grade glioma who received PCV following radiotherapy from Nov 2014 to Nov 2018 wer...
Source: Annals of Oncology - Category: Cancer & Oncology Source Type: research
ConclusionsSurvival of our HGG pts treated into ieCTs compared favorably with a matched CC. Inclusion of HGGs pts into ieCTs should be encouraged. Clinical selection factors predicting which pts may benefit most still lack.Legal entity responsible for the studyThe authors.FundingHas not received any funding.DisclosureM. Simonelli: Advisory / Consultancy: AbbVie. A. Santoro: Advisory / Consultancy: BRISTOL-MYERS-SQUIBB; Advisory / Consultancy: SERVIER; Advisory / Consultancy: GILEAD; Advisory / Consultancy: EISAI; Advisory / Consultancy: BAYER; Advisory / Consultancy: MERCK SHARP&DOHME; Speaker Bureau / Expert testimony...
Source: Annals of Oncology - Category: Cancer & Oncology 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
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