Dysembryoplastic neuroepithelial tumor (DNET/DNT) is a rare WHO grade 1 glioneuronal brain neoplasm and a prototypic low-grade epilepsy-associated neuroepithelial tumor. It typically arises in cortical supratentorial brain, especially temporal and frontal lobes, in children, adolescents, and young adults with long-standing focal seizures. Integrated diagnosis combines clinical epilepsy history, MRI, neuropathology with a specific glioneuronal element, and increasingly molecular testing for FGFR1/RAS-MAPK alterations.
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name: Dysembryoplastic Neuroepithelial Tumor
creation_date: "2026-05-11T14:55:21Z"
updated_date: "2026-05-11T14:55:21Z"
description: >-
Dysembryoplastic neuroepithelial tumor (DNET/DNT) is a rare WHO grade 1
glioneuronal brain neoplasm and a prototypic low-grade epilepsy-associated
neuroepithelial tumor. It typically arises in cortical supratentorial brain,
especially temporal and frontal lobes, in children, adolescents, and young
adults with long-standing focal seizures. Integrated diagnosis combines
clinical epilepsy history, MRI, neuropathology with a specific glioneuronal
element, and increasingly molecular testing for FGFR1/RAS-MAPK alterations.
categories:
- Central Nervous System Neoplasm
- Mixed Neuronal-Glial Tumor
- Epilepsy-Associated Tumor
- Pediatric Brain Tumor
- Low-Grade Glioma
parents:
- mixed neuronal-glial tumor
- brain neoplasm
- structural epilepsy
disease_term:
preferred_term: dysembryoplastic neuroepithelial tumor
term:
id: MONDO:0005505
label: dysembryoplastic neuroepithelial tumor
pathophysiology:
- name: Cortical Glioneuronal Tumor Formation
description: >-
DNET forms as a benign cortical glioneuronal tumor with neuronal and glial
components, often involving temporal or frontal cortex and producing an
epilepsy-associated lesion rather than a high-grade infiltrative glioma.
cell_types:
- preferred_term: neuron
term:
id: CL:0000540
label: neuron
- preferred_term: astrocytic glial component
term:
id: CL:0000127
label: astrocyte
- preferred_term: oligodendrocyte-like glial component
term:
id: CL:0000128
label: oligodendrocyte
locations:
- preferred_term: cerebral cortex
term:
id: UBERON:0000956
label: cerebral cortex
- preferred_term: temporal lobe
term:
id: UBERON:0001871
label: temporal lobe
biological_processes:
- preferred_term: low-grade tumor cell proliferation
modifier: INCREASED
term:
id: GO:0008283
label: cell population proliferation
evidence:
- reference: PMID:37525202
reference_title: "Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
RESULTS: Fourteen cases were reported. There were 12 males and 2 females. Age
range was 9-45 years (mean 19 years). Majority were located in the temporal and
frontal lobes.
explanation: >-
This human case series supports the young age distribution and cortical
temporal/frontal location used for the tumor-formation node.
downstream:
- target: FGFR1-Driven RAS-MAPK Activation
description: Many confirmed DNETs carry FGFR1 alterations that activate MAPK signaling.
- target: Tumor-Associated Cortical Hyperexcitability
description: The cortical glioneuronal lesion is the structural substrate for focal seizures.
- name: FGFR1-Driven RAS-MAPK Activation
description: >-
Specific DNETs are enriched for somatic FGFR1 alterations, including hotspot
mutations, tyrosine kinase-domain duplications, and rare fusions/breakpoints.
These events converge on RAS-MAPK signaling and provide the central molecular
mechanism for many neuropathology-confirmed tumors.
genes:
- preferred_term: FGFR1
term:
id: hgnc:3688
label: FGFR1
gene_products:
- preferred_term: fibroblast growth factor receptor 1
term:
id: NCIT:C17590
label: Fibroblast Growth Factor Receptor 1
biological_processes:
- preferred_term: MAPK cascade
modifier: INCREASED
term:
id: GO:0000165
label: MAPK cascade
evidence:
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
In 43 sporadic cases, in which the diagnosis of DNET could be confirmed on
central blinded neuropathology review, FGFR1 alterations were also frequent
and mainly comprised intragenic tyrosine kinase FGFR1 duplication and multiple
mutants in cis (25/43; 58.1 %) while BRAF p.V600E alterations were absent (0/43).
explanation: >-
The centrally reviewed human tumor cohort supports FGFR1 alteration as the
dominant driver event in confirmed DNET.
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
In conclusion, constitutional and somatic FGFR1 alterations and MAP kinase
pathway activation are key events in the pathogenesis of DNET.
explanation: >-
The authors explicitly connect constitutional/somatic FGFR1 abnormalities
with MAP kinase activation in DNET pathogenesis.
downstream:
- target: Tumor-Associated Cortical Hyperexcitability
description: MAPK-driven glioneuronal tumor formation creates an epileptogenic cortical lesion.
- name: DNET Spectrum Molecular Heterogeneity
description: >-
Specific DNET is molecularly more homogeneous than historical non-specific or
diffuse DNT diagnoses. Broader DNET/MNGT/PLNTY-spectrum tumors can harbor
BRAF, FGFR1, NF1, PDGFRA, FGFR2, NTRK2, and other alterations that converge on
MAPK and PI3K/mTOR signaling, so molecular diagnosis helps separate true DNET
from overlapping low-grade neuroepithelial entities.
genes:
- preferred_term: FGFR1
term:
id: hgnc:3688
label: FGFR1
biological_processes:
- preferred_term: MAPK cascade
modifier: ABNORMAL
term:
id: GO:0000165
label: MAPK cascade
evidence:
- reference: PMID:35836307
reference_title: "The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Specific DNT is a homogeneous group of tumours sharing characteristics of
paediatric low-grade gliomas: a quiet genome with a recurrent genomic alteration
in the RAS-MAPK signalling pathway, a distinct DNA methylation profile and a
good prognosis but showing progression in some cases.
explanation: >-
This integrative human tumor cohort supports separating specific DNT from
heterogeneous non-specific/diffuse tumors and anchors the RAS-MAPK mechanism.
- reference: PMID:31617914
reference_title: Genomic Analysis of Dysembryoplastic Neuroepithelial Tumor Spectrum Reveals a Diversity of Molecular Alterations Dysregulating the MAPK and PI3K/mTOR Pathways.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Overall, DNT-MNGT spectrum tumors exhibit diverse genomic alterations, with
more than half (19/33) leading to MAPK/PI3K pathway alterations.
explanation: >-
This human genomic series supports modeling broader DNT-spectrum molecular
heterogeneity as a MAPK/PI3K-pathway convergent pattern.
- name: Tumor-Associated Cortical Hyperexcitability
description: >-
DNET is an epileptogenic cortical tumor. Seizures arise when the glioneuronal
lesion involves cortical neurons and related networks; peritumoral epileptogenic
zones and associated focal cortical dysplasia can contribute to drug-resistant
focal epilepsy.
cell_types:
- preferred_term: cortical neuron
term:
id: CL:0000540
label: neuron
locations:
- preferred_term: cerebral cortex
term:
id: UBERON:0000956
label: cerebral cortex
evidence:
- reference: PMID:36699536
reference_title: "Low-grade epilepsy-associated neuroepithelial tumors: Tumor spectrum and diagnosis based on genetic alterations."
supports: SUPPORT
evidence_source: OTHER
snippet: >-
Brain tumors can always result in seizures when involving the cortical neurons
or their circuits, and they were found to be one of the most common etiologies
of intractable focal seizures.
explanation: >-
This review supports the cortical-neuron circuit mechanism linking DNET/LEAT
location to intractable focal seizures.
- name: Adjacent Focal Cortical Dysplasia
description: >-
Some DNETs show adjacent cortical dysplasia, representing dual pathology that
can contribute to the epileptogenic peritumoral cortex and surgical planning.
locations:
- preferred_term: cerebral cortex
term:
id: UBERON:0000956
label: cerebral cortex
evidence:
- reference: PMID:37525202
reference_title: "Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Cortical dysplasia was noted in adjacent glial tissue in four cases.
explanation: >-
This case series directly supports adjacent cortical dysplasia as a DNET
dual-pathology finding.
phenotypes:
- category: Neurological
name: Focal-Onset Seizures
description: >-
Long-standing focal seizures are the hallmark clinical presentation and may be
drug-resistant before surgery.
phenotype_term:
preferred_term: Focal-onset seizure
term:
id: HP:0007359
label: Focal-onset seizure
evidence:
- reference: PMID:37525202
reference_title: "Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
BACKGROUND: Dysembryoplastic neuroepithelial tumors are rare benign
supratentotrial epilepsy-associated glioneuronal tumors of children and young
adults. Patients have a long history of seizures.
explanation: >-
The case-series abstract directly supports seizures as the major presentation.
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Dysembryoplastic neuroepithelial tumor (DNET) is a benign brain tumor
associated with intractable drug-resistant epilepsy.
explanation: >-
This tumor genetics paper confirms the association with intractable,
drug-resistant epilepsy.
histopathology:
- name: Specific Glioneuronal Element
description: >-
The diagnostic hallmark is the specific glioneuronal element, with columns of
oligodendrocyte-like cells and floating neurons in a mucinous matrix.
diagnostic: true
evidence:
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The histopathological hallmark of DNET is the specific glioneuronal element,
i.e. the presence of columns of oligodendroglial-like cells and so-called
floating neurons [6].
explanation: >-
This supports the specific glioneuronal element as the diagnostic histologic
hallmark.
- name: Multinodular Pattern
description: >-
Classic DNETs show a multinodular pattern with low proliferative activity and
no high-grade features such as necrosis or significant mitotic activity.
finding_term:
preferred_term: nodular pattern
term:
id: NCIT:C35899
label: Nodular Pattern
evidence:
- reference: PMID:37525202
reference_title: "Clinicopathological features of dysembryoplastic neuroepithelial tumor: a case series."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Histologically, all cases demonstrated a multinodular pattern, specific
glioneuronal component, and floating neurons.
explanation: >-
This human case series supports multinodular architecture and floating
neurons as common DNET histopathologic findings.
diagnosis:
- name: Brain MRI
description: >-
MRI is central to preoperative evaluation and can identify cortical lesion
patterns that support DNET/LEAT diagnosis and surgical planning.
diagnosis_term:
preferred_term: magnetic resonance imaging procedure
term:
id: MAXO:0000424
label: magnetic resonance imaging procedure
results: Cortical tumor location and MRI pattern guide differential diagnosis and surgical planning.
evidence:
- reference: PMID:36672006
reference_title: Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Younger age of seizure onset, bilateral interictal epileptiform discharges,
and MRI type 3 tumors were risk factors for poor prognosis;
explanation: >-
This clinical surgical cohort supports MRI pattern as clinically relevant in
DNET epilepsy prognosis.
- name: Electroencephalography
description: >-
EEG helps localize epileptiform activity and risk-stratify seizure outcomes in
DNET-associated epilepsy.
diagnosis_term:
preferred_term: electroencephalography
term:
id: MAXO:0000932
label: electroencephalography
results: Interictal epileptiform discharges inform localization and outcome prediction.
evidence:
- reference: PMID:36672006
reference_title: Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Younger age of seizure onset, bilateral interictal epileptiform discharges,
and MRI type 3 tumors were risk factors for poor prognosis;
explanation: >-
This supports interictal EEG findings as clinically meaningful in
DNET-associated epilepsy.
- name: Molecular Testing for FGFR1 and MAPK Pathway Alterations
description: >-
Targeted sequencing, fusion testing, and methylation profiling can support
difficult DNET diagnoses and distinguish specific DNT from overlapping
low-grade neuroepithelial tumor entities.
diagnosis_term:
preferred_term: genetic testing
term:
id: MAXO:0000127
label: genetic testing
results: FGFR1 alteration, RAS-MAPK pathway event, or DNT methylation class supports integrated diagnosis.
evidence:
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Here, targeted analysis for FGFR1 hotspot mutations may provide a first step
to aid the diagnosis of DNET and could be supplemented by analyses for FGFR1
fusion transcripts and FGFR1-TKD.
explanation: >-
This supports FGFR1 mutation/fusion/tyrosine-kinase-duplication testing as a
diagnostic adjunct.
- name: DNA Methylation Profiling
description: >-
DNA methylation profiling can help distinguish specific DNT from heterogeneous
non-specific/diffuse DNT-like diagnoses and other low-grade neuroepithelial
tumors.
diagnosis_term:
preferred_term: DNA methylation profiling
results: A distinct DNA methylation profile supports integrated DNT classification.
evidence:
- reference: PMID:35836307
reference_title: "The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
METHODS: We used targeted methods (immunohistochemistry, fluorescence in situ
hybridisation and targeted sequencing) and large-scale genomic methodologies
including DNA methylation profiling to perform an integrative analysis to better
characterise a large retrospective cohort of 82 DNTs, enriched for tumours that
showed progression on imaging.
explanation: >-
This supports DNA methylation profiling as part of integrative diagnostic
characterization for DNT.
genetic:
- name: FGFR1
gene_term:
preferred_term: FGFR1
term:
id: hgnc:3688
label: FGFR1
association: Common somatic driver, with rare familial germline predisposition reported.
relationship_type: CAUSATIVE
variant_origin: GERMLINE_AND_SOMATIC
evidence:
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
By whole-exome sequencing of the familial cases, we identified a novel
germline FGFR1 mutation, p.R661P.
explanation: >-
This supports rare germline FGFR1 predisposition in familial DNET.
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Somatic activating FGFR1 mutations (p.N546K or p.K656E) were observed in the
tumor samples
explanation: >-
This supports somatic activating FGFR1 mutations in tumor tissue.
- reference: PMID:26920151
reference_title: "Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors."
supports: SUPPORT
evidence_source: COMPUTATIONAL
snippet: >-
further evidence for functional relevance was obtained by in silico modeling.
explanation: >-
This separately classifies the in silico modeling component of the FGFR1
functional evidence.
treatments:
- name: Surgical Resection
description: >-
Maximal safe resection or lesionectomy is the main disease-directed therapy,
aiming for tumor control and seizure freedom. Shorter epilepsy duration and
gross total removal predict better seizure outcomes.
treatment_term:
preferred_term: surgical resection
term:
id: MAXO:0000448
label: surgical resection
target_phenotypes:
- preferred_term: Focal-onset seizure
term:
id: HP:0007359
label: Focal-onset seizure
target_mechanisms:
- target: Cortical Glioneuronal Tumor Formation
treatment_effect: MODULATES
description: Resection removes the glioneuronal tumor and associated epileptogenic lesion.
- target: Tumor-Associated Cortical Hyperexcitability
treatment_effect: INHIBITS
description: Resection reduces seizure-generating cortical tumor tissue.
evidence:
- reference: PMID:36672006
reference_title: Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
At the final follow-up, 49 patients (77.8%) were seizure-free.
explanation: >-
This supports favorable seizure outcomes after surgery in a human DNT cohort.
- reference: PMID:36672006
reference_title: Long-Term Seizure Outcomes and Predictors in Patients with Dysembryoplastic Neuroepithelial Tumors Associated with Epilepsy.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Gross total removal of the tumor and a short epilepsy duration were significant
predictors of seizure freedom.
explanation: >-
This supports gross total tumor removal as an important treatment goal.
- reference: PMID:26514362
reference_title: Review of seizure outcomes after surgical resection of dysembryoplastic neuroepithelial tumors.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The median seizure freedom rate was 86% (interquartile range 77-93%) with
only one study reporting fewer than 60% of patients seizure free.
explanation: >-
This systematic review supports high seizure-freedom rates after DNET resection.
- name: Anti-Seizure Medication
description: >-
Anti-seizure medications are used to manage DNET-associated epilepsy, although
seizures are often drug-resistant before definitive surgical management.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: anticonvulsant
term:
id: NCIT:C264
label: Anticonvulsant Agent
target_phenotypes:
- preferred_term: Focal-onset seizure
term:
id: HP:0007359
label: Focal-onset seizure
evidence:
- reference: PMID:26514362
reference_title: Review of seizure outcomes after surgical resection of dysembryoplastic neuroepithelial tumors.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The number of seizure-free patients who discontinued anti-epileptic drugs
varied widely from zero to all patients.
explanation: >-
This supports anti-epileptic drug use as part of clinical management in
surgical DNET epilepsy series.
- name: Mirdametinib MEK Inhibition Trial Therapy
description: >-
Mirdametinib is an investigational brain-penetrant MEK1/2 inhibitor being
evaluated in the SJ901 pediatric/AYA low-grade glioma trial; for DNET, this
is mechanistically relevant to FGFR1-driven RAS-MAPK activation but remains
investigational rather than established standard care.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: mirdametinib
target_mechanisms:
- target: FGFR1-Driven RAS-MAPK Activation
treatment_effect: INHIBITS
description: MEK inhibition targets downstream MAPK pathway signaling activated by FGFR1 alterations.
evidence:
- reference: clinicaltrials:NCT04923126
reference_title: "SJ901: Phase 1/2 Evaluation of Single Agent Mirdametinib (PD-0325901), a Brain-Penetrant MEK1/2 Inhibitor, for the Treatment of Children, Adolescents, and Young Adults With Low-Grade Glioma"
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
This is an open-label, multi-center, Phase 1/2 study of the brain-penetrant
MEK inhibitor, mirdametinib (PD-0325901), in patients with pediatric low-grade
glioma (pLGG).
explanation: >-
The trial record supports an investigational MEK-inhibitor therapy for
pediatric low-grade glioma; DNET relevance is mechanistic and trial-eligibility
based as surfaced by Falcon, so support is marked partial.
clinical_trials:
- name: NCT04923126
phase: PHASE_I
status: RECRUITING
description: >-
SJ901 is an open-label multicenter Phase 1/2 study of mirdametinib, a
brain-penetrant MEK1/2 inhibitor, in pediatric low-grade glioma. Falcon
identified DNET as an eligible LEAT histology in this MAPK-pathway trial.
evidence:
- reference: clinicaltrials:NCT04923126
reference_title: "SJ901: Phase 1/2 Evaluation of Single Agent Mirdametinib (PD-0325901), a Brain-Penetrant MEK1/2 Inhibitor, for the Treatment of Children, Adolescents, and Young Adults With Low-Grade Glioma"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
This is an open-label, multi-center, Phase 1/2 study of the brain-penetrant
MEK inhibitor, mirdametinib (PD-0325901), in patients with pediatric low-grade
glioma (pLGG).
explanation: >-
This exact trial-cache quote supports adding SJ901 as a relevant
low-grade-glioma MEK-inhibitor clinical trial.
notes: >-
ClinicalTrials.gov lists this as a combined Phase 1/2 study; mapped to
PHASE_I because the schema accepts a single phase value. ClinicalTrials.gov
API status checked on 2026-05-11 reported RECRUITING. The corresponding
treatment entry links mirdametinib to FGFR1-Driven RAS-MAPK Activation via
target_mechanisms, because the ClinicalTrial schema does not currently allow
target_mechanisms directly.
datasets:
Target disease: Dysembryoplastic neuroepithelial tumor (also written dysembryoplastic neuroepithelial tumour; abbreviation DNET or DNT). (rahim2023clinicopathologicalfeaturesof pages 1-2, khalilov2024atypicalpresentationof pages 1-2)
Category (high level): WHO-defined circumscribed glioneuronal tumor; a prototypic low-grade epilepsy-associated neuroepithelial tumor (LEAT) entity. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
MONDO ID: Not found in the retrieved primary literature corpus used here (evidence gap noted). (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
| Domain | Key characteristics |
|---|---|
| Definition/classification | • Rare benign glioneuronal tumor; classic representative of low-grade epilepsy-associated tumors (LEATs) • WHO CNS 2021/5th-ed context: WHO grade 1 • Often termed DNET/DNT in literature • Characteristic alteration in LEAT classification tables: FGFR1 (xie2023lowgradeepilepsyassociatedneuroepithelial pages 2-3, xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, xie2023lowgradeepilepsyassociatedneuroepithelial media 10d99c66) |
| Epidemiology | • Rare: estimated incidence ~0.03/100,000/year in the US • Reported prevalence among CNS tumors: ~1.2% in patients <20 years and ~0.2% in >20 years • DNT/DNET is among the commonest LEATs, with ganglioglioma together accounting for >75% of pediatric LEATs and >80% of LEATs in some epilepsy-surgery series (zhang2022longtermseizureoutcomes pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2, pelissier2026pediatriclowgradeepilepsyassociated pages 1-3, iijima2024genotyperelevantneuroimagingfeatures pages 1-2) |
| Typical presentation | • Usually children/adolescents or young adults; LEAT seizure onset often ~12–15 years • Main presentation is chronic focal/drug-resistant epilepsy; seizures may be the only symptom • Rare non-epileptic presentations occur (e.g., headache without epileptiform activity) (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, khalilov2024atypicalpresentationof pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2) |
| Anatomy/location | • Supratentorial, cortical or cortico-subcortical tumor • Strong temporal lobe predilection: ~65–80% of LEATs temporal; >67% temporal in DNT series • Frontal lobe is the second most common site • Frequent association with adjacent focal cortical dysplasia/peritumoral cortical dysplasia (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2, bonney2016reviewofseizure pages 4-5) |
| Imaging/pathology hallmarks | • MRI often shows cortical-based lesion spanning cortical thickness, usually without major mass effect/edema • MRI patterns reported as cystic-like, nodular-like, and dysplastic-like types • Histology: multinodular architecture, specific glioneuronal element, oligodendrocyte-like cells in mucinous matrix, and “floating neurons” • Very low proliferative index; Ki-67 typically <1% (rahim2023clinicopathologicalfeaturesof pages 1-2, zhang2022longtermseizureoutcomes pages 2-5) |
| Molecular genetics | • FGFR1 is the dominant driver: hotspot mutations (p.N546, p.K656), tyrosine kinase domain/internal tandem duplication, and rare fusions • Frequency of FGFR1 alterations reported at ~58.1% in neuropathology-confirmed DNT (25/43) and ~68% in a 58-case specific DNT cohort • MAPK pathway activation is central; some tumors also implicate PI3K/mTOR signaling • BRAF V600E can be reported in the broader DNT spectrum, but was absent in some well-curated specific DNT cohorts; rare alternative fusions include LHFPL3::NTRK2 (rivera2016germlineandsomatic pages 1-2, lucas2020comprehensiveanalysisof pages 1-2, jesus‐ribeiro2022thelandscapeof pages 6-7, pages2022thegenomiclandscape pages 10-10, chen2022casereporta pages 1-2) |
| Diagnostics/molecular testing | • Diagnosis remains integrated: clinical epilepsy history + MRI + neuropathology • Pre-op workup often includes long-term video-EEG; MEG/PET/SEEG used when epileptogenic zone is unclear • Molecular testing increasingly useful for difficult cases: targeted sequencing for FGFR1/BRAF/NTRK alterations and DNA methylation profiling for classification support • sEEG-electrode-derived micro-tissue has been shown feasible for NGS and methylation analysis in a 2024 DNET case (zhang2022longtermseizureoutcomes pages 2-5, gatesman2024characterizationoflow‐grade pages 1-2, xie2023lowgradeepilepsyassociatedneuroepithelial media f56d460d) |
| Treatment/management | • Mainstay is maximal safe surgical resection/lesionectomy, ideally including epileptogenic zone when indicated • Gross-total resection is generally favored for seizure control; lesionectomy alone may suffice in temporal LEATs with normal hippocampus • Anti-seizure medications are used for tumor-related epilepsy, but DNET-associated epilepsy is often pharmacoresistant • Chemo/radiotherapy usually not required for typical indolent DNET; targeted therapy remains investigational/exceptional (takayama2022ishippocampalresection pages 1-2, zhang2022longtermseizureoutcomes pages 1-2, gatesman2024characterizationoflow‐grade pages 1-2) |
| Outcomes/prognosis | • Overall prognosis is favorable; biologically stable tumor with rare malignant transformation • In a 63-patient DNT cohort, 49/63 (77.8%) were seizure-free after surgery; seizure-recurrence-free rates were 82.5% at 2 years, 79.0% at 5 years, and 76.5% at 10 years • Across prior studies, seizure freedom after gross-total resection is often >80%; systematic review median seizure-free range IQR 77–93% • Better outcomes linked to gross-total resection and shorter epilepsy duration; MRI type 3/dysplastic-like pattern and bilateral interictal discharges predict worse seizure outcome (zhang2022longtermseizureoutcomes pages 1-2, zhang2022longtermseizureoutcomes pages 2-5, bonney2016reviewofseizure pages 4-5) |
| Recent developments 2023-2024 | • 2023 LEAT reviews reinforced DNET as a WHO grade 1, FGFR1-linked LEAT entity • 2024 neuroimaging-genotype work showed FGFR1-associated LEAT imaging pattern with 100% sensitivity/specificity in that cohort and poorer seizure-free rates than BRAF-pattern tumors • 2024 S/EEG-based molecular diagnosis showed practical minimally invasive tumor profiling • 2023–2024 literature increasingly emphasizes integrated histology + molecular genetics + methylation classification for diagnostically ambiguous low-grade epilepsy-associated tumors (iijima2024genotyperelevantneuroimagingfeatures pages 1-2, gatesman2024characterizationoflow‐grade pages 1-2, rosemberg2023longtermepilepsyassociatedtumors pages 1-2, rahim2023clinicopathologicalfeaturesof pages 6-7) |
Table: This compact table summarizes core disease-characteristic facts for dysembryoplastic neuroepithelial tumor (DNET/DNT), including classification, phenotype, molecular genetics, diagnosis, treatment, and prognosis. It is designed as a concise knowledge-base-ready snapshot with quantitative details and context-ID citations.
Dysembryoplastic neuroepithelial tumor is a rare, benign/indolent, supratentorial, epilepsy-associated glioneuronal tumor that most commonly affects children and young adults and typically presents with long-standing focal seizures, often drug-resistant. (rahim2023clinicopathologicalfeaturesof pages 1-2, khalilov2024atypicalpresentationof pages 1-2)
Within the LEAT concept (long-term/low-grade epilepsy-associated tumors), DNET is recognized as one of the typical representatives alongside ganglioglioma, with a predilection for neocortical temporal lobe involvement and generally benign growth behavior. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
Multiple WHO-2021 (5th edition) aligned sources list DNET/DNT as WHO grade 1 within LEAT/glioneuronal tumor groupings. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 2-3, xie2023lowgradeepilepsyassociatedneuroepithelial media 10d99c66)
No explicit OMIM/Orphanet/ICD/MeSH/MONDO identifiers were present in the retrieved full-text evidence set. This report therefore does not assert codes without direct supporting evidence. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
Most disease-level information here is derived from aggregated literature sources (reviews, cohorts) plus a few case reports/case series and a clinical registry trial record, rather than EHR-only data. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2, NCT03970785 chunk 1)
Primary causal factors are somatic oncogenic alterations, most commonly involving FGFR1 and the downstream MAPK/ERK pathway, consistent with a developmental/circumscribed tumor biology typical for LEATs. (rivera2016germlineandsomatic pages 1-2, rivera2016germlineandsomatic pages 12-15)
Rivera et al. explicitly conclude that “constitutional and somatic FGFR1 alterations and MAP kinase pathway activation are key events in the pathogenesis of DNET,” supporting a molecularly driven etiology. (rivera2016germlineandsomatic pages 1-2)
Clinical/demographic risk: DNET is predominantly encountered in children/adolescents/young adults (e.g., LEAT seizure onset often around early teens) and occurs supratentorially with temporal predilection, but established population-level environmental risk factors were not identified in the retrieved corpus. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2)
Genetic risk: Most evidence supports somatic drivers rather than inherited predisposition; however, a familial scenario with a germline FGFR1 mutation (p.R661P) plus somatic “second hits” was reported, demonstrating a possible (rare) inherited predisposition mechanism in select families. (rivera2016germlineandsomatic pages 1-2)
No protective genetic variants or environmental protective factors were identified in the retrieved corpus. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
No gene–environment interaction evidence was identified in the retrieved corpus. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
DNET is strongly linked to epilepsy; seizures are typically focal and may be drug-resistant. (rahim2023clinicopathologicalfeaturesof pages 1-2, khalilov2024atypicalpresentationof pages 1-2)
Key phenotypes: * Focal seizures / epilepsy (often drug-resistant) — suggested HPO: HP:0001250 (Seizures); HP:0002197 (Generalized seizures) when present; HP:0007359 (Focal seizures) (term name may vary by HPO release). (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2) * Headache can occur, including rare presentations without epilepsy — suggested HPO: HP:0002315 (Headache). (khalilov2024atypicalpresentationof pages 1-2)
Quantitative examples from a 2023 case series (Pakistan; n=14): age range 9–45 years (mean 19), seizure duration pre-resection 2 months to 9 years (mean 3.2 years), with temporal and frontal lobes most common sites. (rahim2023clinicopathologicalfeaturesof pages 1-2)
Seizures are commonly chronic and may be refractory, motivating epilepsy surgery; thus, DNET can substantially impact function through seizure burden (school/work limitations and medication adverse effects), although disease-specific validated QOL metrics were not extracted from the retrieved corpus. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2, avila2024braintumorrelatedepilepsy pages 10-11)
The dominant molecular theme is RTK → RAS/MAPK/ERK activation, with FGFR1 as a key oncogenic driver in many DNETs; subsets also show alterations converging on PI3K/mTOR. (rivera2016germlineandsomatic pages 1-2, surrey2019genomicanalysisof pages 1-1)
FGFR1 alterations in DNET include: * Hotspot missense mutations (e.g., p.N546K, p.K656E) (rivera2016germlineandsomatic pages 1-2, lucas2020comprehensiveanalysisof pages 1-2) * Tyrosine kinase domain duplication / internal tandem duplication (ITD) (rivera2016germlineandsomatic pages 1-2, pages2022thegenomiclandscape pages 10-10) * Rare fusions/breakpoints involving FGFR1 (rivera2016germlineandsomatic pages 12-15, pages2022thegenomiclandscape pages 10-10)
Somatic vs germline: Rivera et al. provide explicit evidence of both, reporting a germline FGFR1 p.R661P with somatic activating FGFR1 hotspot mutations (p.N546K or p.K656E) in tumor, including a case where p.K656E occurred in cis with the germline variant. (rivera2016germlineandsomatic pages 1-2)
Well-curated DNET cohorts show high enrichment of FGFR1 alterations: * In a neuropathology-confirmed cohort (43 cases), FGFR1 alterations were frequent and “mainly comprised intragenic tyrosine kinase FGFR1 duplication and multiple mutants in cis (25/43; 58.1%) while BRAF p.V600E alterations were absent (0/43).” (rivera2016germlineandsomatic pages 1-2) * In a cohort of 58 “specific” DNTs, Pagès et al. report FGFR1 disruption (mutation, ITD, fusion) and state “In our cohort of 58 specific DNTs, we found a similar frequency (68%)” (of FGFR1-related events). (pages2022thegenomiclandscape pages 10-10)
Broader DNT/MNGT spectrum sequencing shows that alterations frequently converge on MAPK/PI3K signaling; in one series, “more than half” (19/33) of analyzed tumors had alterations predicted to dysregulate MAPK and/or PI3K pathways. (surrey2019genomicanalysisof pages 1-1)
DNA methylation profiling is increasingly used for difficult-to-classify low-grade neuroepithelial tumors and can support/refine diagnosis beyond morphology alone; however, DNET-specific methylation subclass statistics were not available from the retrieved evidence set. (gatesman2024characterizationoflow‐grade pages 1-2)
No validated environmental/lifestyle/infectious causal factors were identified for DNET in the retrieved corpus. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
A commonly supported mechanistic chain is: 1) FGFR1 activation (hotspot mutation, ITD/kinase duplication, or fusion) → 2) increased MAPK/ERK signaling (phospho-ERK upregulation) → 3) tumor formation/maintenance in cortex (glioneuronal tumor microenvironment) → 4) cortical network hyperexcitability and epileptogenesis, clinically manifesting as focal seizures and drug-resistant epilepsy; seizure burden is also influenced by the epileptogenic zone extending into peritumoral cortex and co-existing focal cortical dysplasia (“dual pathology”). (rivera2016germlineandsomatic pages 12-15, xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, bonney2016reviewofseizure pages 4-5)
Rivera et al. provide functional support for MAPK activation: phospho-ERK was upregulated in FGFR1-mutated cases and they report immunohistochemical confirmation of phospho-ERK upregulation in “24/35 (69%)” of FGFR1-mutated cases. (rivera2016germlineandsomatic pages 12-15)
The tumor is glioneuronal, implicating glial-lineage and neuronal components and peritumoral neuronal circuitry.
Suggested Cell Ontology (CL) terms (examples): * CL:0000540 (Neuron) for epileptogenic network effects and “floating neurons” concept (pathology context). (rahim2023clinicopathologicalfeaturesof pages 1-2) * CL:0000127 (Astrocyte) and CL:0000128 (Oligodendrocyte) as relevant to glial components/oligodendrocyte-like cells described histologically. (rahim2023clinicopathologicalfeaturesof pages 1-2)
Suggested GO Biological Process terms (examples): * MAPK cascade (e.g., GO:0000165) and ERK1/2 cascade (relevant to phospho-ERK evidence). (rivera2016germlineandsomatic pages 12-15) * Regulation of synaptic transmission / neuronal excitability (epileptogenesis context; general). (avila2024braintumorrelatedepilepsy pages 10-11)
Primary system affected is the central nervous system (brain), with seizures as the dominant symptom. (rahim2023clinicopathologicalfeaturesof pages 1-2, xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2)
DNET is typically supratentorial and cortical with strong temporal lobe predominance and frequent frontal involvement. (rahim2023clinicopathologicalfeaturesof pages 1-2, xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2)
Suggested UBERON terms (examples): * UBERON:0000955 (brain) * UBERON:0001870 (cerebral cortex) * UBERON:0002285 (temporal lobe) * UBERON:0001871 (frontal lobe)
A frequent associated lesion is focal cortical dysplasia adjacent to tumor (dual pathology), implicating both tumor and surrounding cortex. (khalilov2024atypicalpresentationof pages 1-2)
DNETs are described as occurring most often in childhood/adolescence; LEAT seizures commonly begin in early teens and frequently precede surgery by years. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, rahim2023clinicopathologicalfeaturesof pages 1-2)
Tumors are usually slow-growing/indolent with long seizure histories; malignant transformation is rare. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2, zhang2022longtermseizureoutcomes pages 1-2)
Quantitative epidemiology is limited but a large surgical cohort review reports DNET incidence around 0.03/100,000/year (US estimate) and highlights that DNET constitutes a substantial fraction of LEAT diagnoses in epilepsy surgery series. (zhang2022longtermseizureoutcomes pages 1-2, iijima2024genotyperelevantneuroimagingfeatures pages 1-2)
DNET is primarily driven by somatic alterations, but rare familial predisposition via germline FGFR1 alteration has been reported. (rivera2016germlineandsomatic pages 1-2)
Diagnosis is typically based on seizure history plus MRI and confirmatory histopathology; preoperative epilepsy workup commonly includes long-term video-EEG, with additional modalities (MEG, PET, invasive monitoring) when needed to define the epileptogenic zone. (zhang2022longtermseizureoutcomes pages 2-5, takayama2022ishippocampalresection pages 1-2)
MRI is emphasized as the key modality; lesions are often cortical-based and may be classified into morphologic patterns (e.g., cystic-like, nodular-like, dysplastic-like). (zhang2022longtermseizureoutcomes pages 2-5, rahim2023clinicopathologicalfeaturesof pages 1-2)
A 2024 radiogenomic study in LEATs showed that certain imaging patterns can predict genotype with high accuracy, reporting imaging groups with “93.8% sensitivity and 100% specificity to BRAF V600E” (Group 1) and “100% sensitivity and specificity for FGFR1 mutations” (Group 2). (iijima2024genotyperelevantneuroimagingfeatures pages 1-2)
Characteristic pathology includes a multinodular architecture and “floating neurons” within a specific glioneuronal element; Ki-67 is typically very low. (rahim2023clinicopathologicalfeaturesof pages 1-2)
Molecular profiling supports diagnosis and may clarify difficult cases, particularly in the DNT/MNGT/PLNTY spectrum where morphology overlaps; testing may include targeted DNA/RNA sequencing for FGFR1/BRAF/NTRK alterations and methylation arrays. (surrey2019genomicanalysisof pages 1-1, gatesman2024characterizationoflow‐grade pages 1-2)
A 2024 proof-of-concept study demonstrated feasibility of extracting tumor DNA from tissue adherent to stereoelectroencephalography (sEEG) electrodes and performing targeted sequencing and DNA methylation array analysis to aid classification. (gatesman2024characterizationoflow‐grade pages 1-2)
In a 63-patient DNET cohort (2008–2021), 49/63 (77.8%) were seizure-free after surgery, with cumulative seizure recurrence-free rates 82.5% (2 years), 79.0% (5 years), 76.5% (10 years). (zhang2022longtermseizureoutcomes pages 1-2)
A systematic review of surgical series reported a median seizure-freedom rate of 86% (IQR 77–93%) and highlighted that gross-total resection is repeatedly associated with seizure freedom. (bonney2016reviewofseizure pages 4-5)
Predictors of better seizure outcomes include gross total resection and shorter epilepsy duration, while certain imaging patterns (e.g., dysplastic-like) and bilateral interictal epileptiform discharges may predict poorer seizure outcomes. (zhang2022longtermseizureoutcomes pages 1-2)
Maximal safe surgical resection/lesionectomy is widely considered the optimal approach to achieve seizure control and durable tumor control in LEATs, including DNET. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2)
A hippocampus-sparing strategy may be appropriate in selected temporal-lobe LEATs with normal hippocampus: in a cohort of 32 temporal LEAT cases, 28/32 (87.5%) achieved seizure freedom irrespective of hippocampal resection, and additional hippocampal resection negatively impacted verbal outcomes. (takayama2022ishippocampalresection pages 1-2)
Real-world implementation example: an observational clinical registry study evaluated intraoperative fluorescein-guided resection feasibility for ganglioglioma and DNET (NCT03970785), using surgical video review and postoperative MRI to assess fluorescence and extent of resection, with Engel-classification seizure outcomes. (NCT03970785 chunk 1)
DNET-associated epilepsy is frequently treated with ASMs but may be drug-resistant; consensus neuro-oncology practice avoids routine prophylactic ASM use in seizure-naïve patients. (rahim2023clinicopathologicalfeaturesof pages 1-2, avila2024braintumorrelatedepilepsy pages 10-11)
A 2024 Society for Neuro-Oncology consensus review states prophylactic ASM in seizure-naïve brain tumor patients lacks high-quality evidence, yet ~70% of neurosurgeons give a short postoperative course (commonly levetiracetam for 7 days after supratentorial craniotomy); meta-analysis showed reduced early postoperative seizures (RR 0.35, 95% CI 0.13–0.95) but not late seizures. (avila2024braintumorrelatedepilepsy pages 10-11)
Newton & Wojkowski (2024) summarize that seizures affect >50% of brain tumor patients overall and provide tumor-specific estimates (DNET ~100%), and similarly advise that prophylactic AEDs are not recommended; after a first verified seizure, consensus is to start ASM monotherapy, most often levetiracetam (with alternatives/add-ons including lacosamide, valproate, brivaracetam, lamotrigine, and perampanel). (newton2024antiepilepticstrategiesfor pages 1-3)
Because DNET biology is frequently MAPK-driven (FGFR1 alterations, RTK fusions), MEK-pathway targeting is being evaluated within broader pediatric/AYA low-grade glioma frameworks.
Clinical trial example (MAPK-pathway therapy): SJ901 (NCT04923126) evaluates mirdametinib (MEK1/2 inhibitor) in pediatric/AYA low-grade glioma and explicitly includes LEAT histologies such as dysembryoplastic neuroepithelial tumor (DNET) among eligible conditions; a separate protocol chunk specifies requirement for centrally reviewed MAPK-pathway activation evidence (including FGFR1/2/3 aberrations and other RAS/MAPK genes). (NCT04923126 chunk 2, NCT04923126 chunk 4)
Because MAXO codes were not present in the evidence corpus, the following are suggested as likely appropriate mappings (to be verified against MAXO): * Neurosurgical resection of brain tumor (lesionectomy/gross total resection). (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2) * Antiseizure medication therapy (levetiracetam monotherapy; add-on therapy). (avila2024braintumorrelatedepilepsy pages 10-11) * Stereoelectroencephalography (SEEG) monitoring / intracranial EEG evaluation. (gatesman2024characterizationoflow‐grade pages 1-2) * Intraoperative fluorescence-guided surgery. (NCT03970785 chunk 1) * MEK inhibitor therapy (mirdametinib clinical trial context). (NCT04923126 chunk 2)
No primary-prevention interventions are established for DNET based on available evidence; secondary prevention in practice is largely early recognition of epileptogenic lesions and timely referral for epilepsy surgery evaluation when seizures are drug-resistant. (xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2)
No naturally occurring DNET analogue in non-human species was identified in the retrieved corpus. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2)
A 2024 review on epilepsy-associated tumors notes improving animal modeling capacity for low-grade neuroepithelial tumors (LGNTs) with tools such as in utero electroporation to generate tumors with relevant genetic features; however, DNET-specific validated model organism details were not extracted from the retrieved evidence set. (rahim2023clinicopathologicalfeaturesof pages 6-7)
Recent LEAT-focused reviews emphasize that DNET is a core LEAT entity and that the WHO CNS 2021 classification recognized additional epilepsy-associated tumor entities, increasing the importance of integrated diagnosis using histology plus molecular tools. (rosemberg2023longtermepilepsyassociatedtumors pages 1-2, xie2023lowgradeepilepsyassociatedneuroepithelial pages 1-2)
A WHO-2021-based LEAT table explicitly lists DNET as WHO grade 1 with characteristic genetic alteration FGFR1, supporting a molecularly anchored diagnostic approach rather than morphology alone. (xie2023lowgradeepilepsyassociatedneuroepithelial media 10d99c66)
The 2024 radiogenomic study provides a practical framework suggesting that preoperative neuroimaging patterns can predict genotype, potentially enabling earlier precision planning (e.g., anticipating FGFR1 vs BRAF-driven lesions), and also indicates seizure outcomes may differ by genotype-associated imaging group. (iijima2024genotyperelevantneuroimagingfeatures pages 1-2)
A 2024 proof-of-concept sEEG-electrode approach suggests that neurosurgical epilepsy workflows can also become molecular diagnostic workflows, enabling targeted sequencing and methylation analysis from microscopic tissue without requiring a separate biopsy procedure. (gatesman2024characterizationoflow‐grade pages 1-2)
The 2024 SNO consensus emphasizes evidence-based antiseizure management principles that generalize to DNET patients (especially perioperative decisions): no strong support for routine prophylaxis in seizure-naïve cases, frequent use of short postoperative levetiracetam courses, preference for monotherapy and non–enzyme-inducing ASMs, and escalation toward local therapies (surgery/irradiation) for drug-resistant seizures. (avila2024braintumorrelatedepilepsy pages 10-11)
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(NCT03970785 chunk 1): Intraoperative Fluorescence of Ganglogliomas and Neuroepithelial Dysembryoplastic Tumors. Rennes University Hospital. 2018. ClinicalTrials.gov Identifier: NCT03970785
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(NCT04923126 chunk 2): SJ901: Evaluation of Mirdametinib in Children, Adolescents, and Young Adults With Low-Grade Glioma. St. Jude Children's Research Hospital. 2021. ClinicalTrials.gov Identifier: NCT04923126
(NCT04923126 chunk 4): SJ901: Evaluation of Mirdametinib in Children, Adolescents, and Young Adults With Low-Grade Glioma. St. Jude Children's Research Hospital. 2021. ClinicalTrials.gov Identifier: NCT04923126