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CANCER:

Ganglioglioma

1d
Neuropathology and epilepsy surgery - 2024 update. (PubMed, Free Neuropathol)
Finally, I will highlight the ongoing discussion addressing commonalities between temporal lobe epilepsy and Alzheimer's disease, the impact of adult neurogenesis and gliogenesis for the initiation and progression of temporal lobe seizures in the human brain as well as the immunopathogenesis of glutamic acid decarboxylase antibody associated temporal lobe epilepsy as a meaningful disease entity. This review will update the reader on some of these fascinating publications from 2022 and 2023 which were selected carefully, yet subjectively, by the author.
Journal • Surgery
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BRAF (B-raf proto-oncogene) • PTPN11 (Protein Tyrosine Phosphatase Non-Receptor Type 11) • SLC35A2 (Solute Carrier Family 35 Member A2)
7d
Trial completion date
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PTCH1 (Patched 1)
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PTCH1 mutation
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Mekinist (trametinib) • gemcitabine • Kisqali (ribociclib) • Odomzo (sonidegib) • Neupogen (filgrastim)
3ms
Long-term epilepsy associated-tumors (LEATs): what is new? (PubMed, Arq Neuropsiquiatr)
Some of them such as diffuse astrocytoma MIB or MYBL1 altered, polymorphous low-grade neuroepithelial tumor of the young (PLNTY), and multilocular and vacuolating neuronal tumor (MVNT) are currently considered LEATs. The relationship between LEATs and epilepsy is still a matter of debate, and there is a general agreement about the beneficial effects of an early neurosurgical intervention on the clinical outcome.
Journal
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MYBL1 (MYB Proto-Oncogene Like 1)
3ms
Germline BRCA2 pathogenic variants in pediatric ganglioglioma: Case report and review of the literature. (PubMed, Childs Nerv Syst)
BRCA2 germline variants may increase the risk of developing some types of pediatric brain tumors, but further study is needed to determine its effect on low-grade glioma formation.
Review • Journal • BRCA Biomarker
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BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset)
3ms
Trial completion date
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PTCH1 (Patched 1)
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PTCH1 mutation
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Mekinist (trametinib) • gemcitabine • Kisqali (ribociclib) • Odomzo (sonidegib) • Neupogen (filgrastim)
4ms
Social Determinants of Health in Glioblastoma Population (clinicaltrials.gov)
P=N/A, N=100, Completed, Wake Forest University Health Sciences | Active, not recruiting --> Completed
Trial completion
4ms
Leat-associated seizures the possible role of EAAT2, pyruvate carboxylase and glutamine synthetase. (PubMed, Epilepsy Res)
The retained expression of pyruvate carboxylase may contribute to determining a pathological glutamate excess unopposed by glutamine synthetase that resulted expressed to a variable extent in the majority of the tumors. Furthermore, we can assume that the EAAT2 loss in brain tumors in general and in LEATs in particular is more conceivably epigenetic.
Journal
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MYBL1 (MYB Proto-Oncogene Like 1) • SLC1A2 (Solute Carrier Family 1 Member 2)
4ms
Evaluation of Hippocampal-Avoidance Using Proton Therapy in Low-Grade Glioma (clinicaltrials.gov)
P=N/A, N=74, Recruiting, St. Jude Children's Research Hospital | Phase classification: P2 --> P=N/A | Trial primary completion date: Jul 2023 --> Jul 2027
Phase classification • Trial primary completion date
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BRAF (B-raf proto-oncogene)
4ms
A new subtype of diffuse midline glioma, H3 K27 and BRAF/FGFR1 co-altered: a clinico-radiological and histomolecular characterisation. (PubMed, Acta Neuropathol)
Contrary to other DMG, these tumours occur more frequently in the thalamus (70% for BRAF and 58% for FGFR1) and patients have a longer overall survival with a median above three years. In conclusion, DMG, H3 K27 and BRAF/FGFR1 co-altered represent a new subtype of DMG with distinct genotype/phenotype characteristics, which deserve further attention with respect to trial interpretation and patient management.
Journal
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BRAF (B-raf proto-oncogene) • FGFR1 (Fibroblast growth factor receptor 1) • H3-3A (H3.3 Histone A)
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BRAF mutation • H3.3K27M
4ms
Ganglioglioma cells potentiate neuronal network synchronicity and elicit burst discharges via released factors. (PubMed, Neurobiol Dis)
Taken together, we demonstrate that GG-derived paracrine signaling alone is sufficient to induce accelerated neuronal network development accompanied by astrocytic proliferation. Perspectively, a deeper understanding of factors involved may serve as the basis for future therapeutic approaches.
Journal
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BRAF (B-raf proto-oncogene) • TP53 (Tumor protein P53) • S100A8 (S100 Calcium Binding Protein A8) • APOE (Apolipoprotein E)
4ms
Epilepsy Outcome and Pathology Analysis for Ganglioglioma: A Series of 51 Pediatric Patients. (PubMed, Pediatr Neurol)
The overall outcome of GG and epilepsy in children is optimistic, and the outcome is not closely related to the presence of BRAF V600E mutation and CD34 (+). The FCD surrounding GG could be type I or type II. Incomplete resection of the surrounding FCD has the risk of unsatisfactory control of epilepsy. Children with the BRAF V600E mutation may be prone to early-onset epilepsy. The expression of CD34 is more likely to be detected in children with older age and a long duration of epilepsy.
Journal
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BRAF (B-raf proto-oncogene) • CD34 (CD34 molecule)
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BRAF V600E • BRAF V600
4ms
Polymorphous Low Grade Neuroepithelial Tumor of the Young: An Institutional Case Series with Radiological and Pathological Correlation (RSNA 2023)
6) Surgical resection is usually curative of seizures. *Table of Contents/Outline: -Introduction, PLNTY’s place in the WHO 2021 Classification of CNS tumors -Imaging characteristics of PLNTY -Histological characteristics of PLNTY -Discussion of pathophysiology and molecular subtypes -Imaging comparisons of differential diagnoses
Clinical
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BRAF (B-raf proto-oncogene) • FGFR2 (Fibroblast growth factor receptor 2) • FGFR3 (Fibroblast growth factor receptor 3) • CD34 (CD34 molecule)
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BRAF V600E • BRAF V600 • FGFR2 mutation
5ms
Nlrp3+ tumor-associated myeloid cells are enriched in astrocytic gliomas and represent a therapeutic vulnerability (SNO 2023)
1) Astrocytomas (IDHMut-non-codel) comprise TAM subsets common across multiple tumor types. 2) The FOLR2+ TAMs predict favorable outcomes, while the NLRP3+ TAMs are immunosuppressive and predict adverse outcomes in breast cancer. Given the inverse expression of NLRP3 and FOLR2 in astrocytomas, we hypothesize that NLRP3 TAMs+ are immunosuppressive and a potential immunotherapeutic target in astrocytomas.
IO biomarker
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • SPP1 (Secreted Phosphoprotein 1) • NLRP3 (NLR Family Pyrin Domain Containing 3)
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IDH1 mutation
5ms
Efficacy of BRAF inhibitor plixorafenib (FORE8394) in recurrent, primary central nervous system tumors (PCNST) (SNO 2023)
An approved BRAFi, when used with MEKi, has ORR 33% with mDOR 13.6=months and ORR=50% with DOR of 6-29 months in V600+ HGG and LGG, respectively (dabrafenib US Prescribing Information, 2023). Phase 1/2a is a single-arm study (NCT02428712) in patients (n=113) with BRAF-altered advanced tumors to assess safety, PK, and efficacy of oral plixorafenib 900-3600 mg/day alone or with cobicistat (CYP3A4/P-gp inhibitor)...Prior anti-cancer treatments included surgery (8/10), radiotherapy (8/10), and systemic therapy (8/10, including temozolomide [n=8], bevacizumab [n=2], carboplatin [n=1], dendritic cell vaccine [n=1])...Plixorafenib has a benign safety profile and leads to a high ORR and durable response in MAPKi-naïve BRAFV600+ PCNST. A phase 2 study is ongoing to confirm these findings (NCT05503797).
Clinical
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BRAF (B-raf proto-oncogene) • CYP3A4 (Cytochrome P450, family 3, subfamily A, polypeptide 4)
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Avastin (bevacizumab) • Tafinlar (dabrafenib) • carboplatin • temozolomide • plixorafenib (FORE-8394) • Tybost (cobicistat)
5ms
Long term activity of vemurafenib in cancers with BRAF mutations: the ACSE basket study for advanced cancers other than BRAF-mutated melanoma. (PubMed, ESMO Open)
Responses and prolonged PFS were observed in many tumours with BRAF mutations, including HCL, ECD, ovarian carcinoma, gliomas, ganglioglioma, and sarcomas. Although not all cancer types responded, vemurafenib is an agnostic oncogene therapy of cancers.
Journal • Pan tumor • Metastases
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BRAF (B-raf proto-oncogene)
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BRAF mutation
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Zelboraf (vemurafenib)
5ms
Social Determinants of Health in Glioblastoma Population (clinicaltrials.gov)
P=N/A, N=100, Active, not recruiting, Wake Forest University Health Sciences | Recruiting --> Active, not recruiting
Enrollment closed
6ms
Diagnostic accuracy of anti-3-[F]-FACBC PET/MRI in gliomas. (PubMed, Eur J Nucl Med Mol Imaging)
Anti-3-[F]FACBC PET demonstrated high uptake in the majority of gliomas, especially in IDH wildtype gliomas, and improved the accuracy of preoperatively predicted glioma diagnoses.
Journal
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IDH1 (Isocitrate dehydrogenase (NADP(+)) 1) • IDH2 (Isocitrate Dehydrogenase (NADP(+)) 2)
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IDH wild-type
6ms
Nomograms Based on MRI Radiomics for Differential Diagnosis and Predicting BRAFV600E Expression in Pleomorphic Xanthoastrocytoma and Ganglioglioma. (PubMed, Acad Radiol)
Nomograms based on radiomics and clinical semantic features were noninvasive tools for differential diagnosis of PXA and GG and predicting BRAFV600E expression, which may be helpful for assessing patient prognosis and developing individualized treatment strategies.
Journal
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BRAF V600E • BRAF V600
6ms
Unusual Locations of Gangliogliomas: Intraventricular and Posterior Fossa. (PubMed, Turk Neurosurg)
GG should be considered in the differential diagnosis of patients with tumors in the intraventricular region or posterior fossa. Maximal tumor resection and restoration of CSF flow pathways ensure a good outcome. Growth patterns correlate with resection and can help choose the best candidates for surgery. However, further studies on large patient samples are needed.
Retrospective data • Journal
7ms
Surgical treatment of epilepsy - Initial experience from a comprehensive epilepsy program in coastal South India. (PubMed, J Neurosci Rural Pract)
In carefully selected patients with drug-resistant epilepsy, surgery offers an excellent chance of becoming seizure-free with significant improvement in overall quality of life. Majority of the common epilepsy surgery procedures can be performed through a multidisciplinary approach even in centers with limited resources.
Journal
7ms
Comparison of Imaging Characteristics of Gangliogliomas between Child/Adolescent Group and Adult Group. (PubMed, Curr Med Imaging)
Peri-tumoral edema was significantly less prevalent in the child/adolescent group, whereas marked enhancement was significantly more frequent in the adult group. To ensure accurate results, a larger case series should be conducted to validate our findings.
Journal
7ms
Intraoperative visual evoked potential recording in occipital tumor surgery (PubMed, Zh Vopr Neirokhir Im N N Burdenko)
MR tractography of optic radiation and intraoperative monitoring of cVEP allow choosing the safest approach for resection of occipital tumor and minimizing the risk of damage to visual cortex and optic radiation fibers. In most cases, postoperative visual functions do not worsen after intraoperative mapping of visual cortex and determining the safest trajectory for resection of occipital lobe tumors. Moreover, improvement is observed in some cases.
Journal • Surgery
7ms
Recurrent TRAK1::RAF1 Fusions in pediatric low-grade gliomas. (PubMed, Brain Pathol)
The remaining tumor was non-classifiable; with focal recurrence 14 months after initial resection; the patient remains symptom free and without further recurrence/progression (5 months post re-resection and 19 months from initial diagnosis). Our report expands the landscape of oncogenic RAF1 fusions in pediatric gliomas, which will help to further refine tumor classification and guide management of patients with these alterations.
Journal
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RAF1 (Raf-1 Proto-Oncogene Serine/Threonine Kinase)
7ms
A comprehensive analysis of infantile central nervous system tumors to improve distinctive criteria for infant-type hemispheric glioma versus desmoplastic infantile ganglioglioma/astrocytoma. (PubMed, Brain Pathol)
Moreover, these results are consistent with the literature data concerning the molecular dichotomy (BRAF/RAF1 alterations vs. RTK genes' fusions) between DIG/DIA and IHG. This study characterized histopathologically and radiologically two additional cases of the novel embryonal tumor characterized by PLAGL2 gene amplification.
Journal
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BRAF (B-raf proto-oncogene) • ALK (Anaplastic lymphoma kinase) • ROS1 (Proto-Oncogene Tyrosine-Protein Kinase ROS) • NTRK1 (Neurotrophic tyrosine kinase, receptor, type 1) • NTRK3 (Neurotrophic tyrosine kinase, receptor, type 3) • NTRK2 (Neurotrophic tyrosine kinase, receptor, type 2) • RAF1 (Raf-1 Proto-Oncogene Serine/Threonine Kinase) • YAP1 (Yes associated protein 1) • GNLY (Granulysin) • ZFTA (Zinc Finger Translocation Associated) • PLAGL2 (PLAG1 Like Zinc Finger 2)
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NTRK1 fusion • NTRK2 fusion • ALK fusion • ROS1 fusion
7ms
The integrated genomic and epigenomic landscape of gangliogliomas - retrospective analysis of a single-centre case series (ECP 2023)
One case presented with concerning copy number changes and TERT promoter mutation, raising the possibility of glioblastoma. Further studies regarding the biological behaviour of these tumours are needed.
Retrospective data
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KRAS (KRAS proto-oncogene GTPase) • BRAF (B-raf proto-oncogene) • FGFR2 (Fibroblast growth factor receptor 2) • FGFR3 (Fibroblast growth factor receptor 3) • NTRK2 (Neurotrophic tyrosine kinase, receptor, type 2) • TERT (Telomerase Reverse Transcriptase) • KIAA1549 • SHTN1 (Shootin 1)
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BRAF V600E • KRAS mutation • BRAF V600 • NTRK2 fusion • FGFR2 mutation • BRAF wild-type • FGFR2 fusion • KIAA1549-BRAF fusion • BRAF fusion • FGFR3 fusion • TERT mutation • TERT promoter mutation • KRAS Q61K
7ms
Robot-assisted transcerebellar stereotactic approach to the posterior fossa in pediatric patients: a technical note. (PubMed, Childs Nerv Syst)
According to our experience, the transcerebellar frame-based robotic stereotactic approach to the cerebellum and the brainstem is feasible, safe, and effective even in young children.
Journal
7ms
A unique case of intracranial collision tumor composed of ganglioglioma WHO gr I and supratentorial ependymoma WHO gr III: case-based literature review. (PubMed, Childs Nerv Syst)
To our best knowledge, no previous reports of collision tumor composed of ganglioglioma and supratentorial ependymoma in a single patient have been reported. We believe that this report could significantly contribute to further surgical practice as well as to treatment decision for these types of collision tumors.
Review • Journal
8ms
Long-term outcomes of symptomatic optic pathway glioma: 32-year experience at a single Western Australian tertiary pediatric oncology center. (PubMed, Front Oncol)
Three patients treated with radiotherapy developed second brain tumors. 25-year OS in this cohort was 77.5% but survivorship carried significant long-term morbidities including radiation-induced second malignant brain tumors.
Journal
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NF1 (Neurofibromin 1)
8ms
Vestibular seizures and spontaneous downbeat nystagmus of ganglioglioma origin: a case report. (PubMed, BMC Neurol)
Asides from the cortical pathogenesis of epileptic vertigo, this case also provides insight into the DBN secondary to tumor of the temporal lobe. Moreover, the 24-h EEG is advantageous to recognize vestibular seizures and localize the ictal onset areas.
Journal
8ms
Benign Spinal Tumors. (PubMed, Adv Exp Med Biol)
This goal is more achievable with intradural extramedullary tumors; yet, with a meticulous surgical approach, many of the intramedullary tumors are amenable for safe gross-total or near-total resection. The nature of these tumors is benign; hence, a different way to measure outcome success is pursued and usually depends on functional rather than oncological or survival outcomes.
Journal
8ms
Benign Glioma. (PubMed, Adv Exp Med Biol)
Also discussed is ganglioglioma (GG), a mixed neuronal-glial tumor that represents a notable diagnosis in the differential for other LGG (Wesseling and Capper 2018). Ependymomas of the brain and spinal cord, including major histologic subtypes, are discussed in other chapters.
Journal
9ms
A rare tumor in the sellar region: ganglioglioma, a case report and a general overview. (PubMed, Childs Nerv Syst)
Complete tumor resection may not be feasible in sellar region gangliogliomas, especially in pediatric cases, due to endocrinological and vision-related complications. In cases where complete resection is not possible, radiotherapy and/or chemotherapy may be considered. However, the optimal treatment approach has not yet been established, and further research is needed.
Journal
9ms
MRI characteristics predict BRAF V600E status in gangliogliomas and pleomorphic xanthoastrocytomas and provide survival prognostication. (PubMed, Acta Radiol)
Imaging features are potentially predictive of BRAF V600E status in GGs and PXAs. Furthermore, rADC value is a valuable prognostic factor for patients with GGs or PXAs.
Journal
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BRAF (B-raf proto-oncogene)
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BRAF V600E • BRAF V600
9ms
Social Determinants of Health in Glioblastoma Population (clinicaltrials.gov)
P=N/A, N=115, Recruiting, Wake Forest University Health Sciences | Trial completion date: Jun 2023 --> Oct 2023 | Trial primary completion date: Jun 2023 --> Oct 2023
Trial completion date • Trial primary completion date
10ms
The utility of methylation profiling in the diagnosis of pediatric BRAF mutant glial and glioneuronal tumors (AANP 2023)
Conclusions In BRAF mutants glial or glioneuronal tumors, MP modified the diagnosis in 2/46 cases and raised the debate of GG vs. PA in a subset of cases. However, it did not resolve the challenging cases, and the MP of PXA should be interpreted with caution.
Clinical
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BRAF (B-raf proto-oncogene)
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BRAF mutation