ArticleNeurology2020
Nonoptic pathway tumors in children with neurofibromatosis type 1.
Article in Neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 22 citations in OpenAlex.
- Bridging pediatric and adult neuro-oncology: Insights into adolescents and young adults (AYA) central nervous system tumors.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2026Review
- Clinical characteristics and treatment strategies of brainstem lesion in children with neurofibromatosis type 1.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Current states in understanding oligodendroglia-mediated neurological issues in neurofibromatosis type 1 (NF1).Acta neuropathologica communications · 2025Review
- Non-Optic Glioma-like Lesions in Adult Neurofibromatosis Type 1 Patients.Diagnostics (Basel, Switzerland) · 2024Article
- Low-grade glioma in children with neurofibromatosis type 1: surveillance, treatment indications, management, and future directions.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Review
- Clinical, histological, and molecular features of gliomas in adults with neurofibromatosis type 1.Neuro-oncology · 2023Article
- MRI-guided laser interstitial thermal therapy for deep-seated gliomas in children with neurofibromatosis type 1: report of two cases.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Article
- ERN GENTURIS tumour surveillance guidelines for individuals with neurofibromatosis type 1.EClinicalMedicine · 2023Review
- Dose-dependent seizure control with MEK inhibitor therapy for progressive glioma in a child with neurofibromatosis type 1.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Article
- The Current State of Radiotherapy for Pediatric Brain Tumors: An Overview of Post-Radiotherapy Neurocognitive Decline and Outcomes.Journal of personalized medicine · 2022Review
- Disseminated craniospinal low-grade glioma in a patient with NF-1 without optic pathway pathology: illustrative case.Journal of neurosurgery. Case lessons · 2021Article
- Radiohistogenomics of pediatric low-grade neuroepithelial tumors.Neuroradiology · 2021Review
- Clinical characterization of children and adolescents with NF1 microdeletions.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Article
- Children with supratentorial midline pilocytic astrocytomas exhibit multiple progressions and acquisition of neurologic deficits over time.Neuro-oncology advancesArticle
- Location and extent of disease predicts outcome of neurofibromatosis type 1-related pediatric low-grade gliomas.Neuro-oncology advancesArticle
- Emerging mechanism and therapeutic potential of neurofibromatosis type 1-related nerve system tumor: Advancing insights into tumor development.Neuro-oncology advancesReview
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo define the radiologic features and natural history of nonoptic pathway tumors (non-OPTs) in children with neurofibromatosis type 1 (NF1).
methodsWe performed a retrospective cross-sectional analysis of 64 children with NF1 harboring 100 probable non-OPTs. Age at diagnosis, sex, tumor location, number of tumors, symptomology, concurrent OPT, radiographic progression (defined as qualitative and quantitative increases in size), and treatment were assessed. Tumor volumes were measured from initial presentation until treatment or end of disease progression.
resultsSixty-three percent of probable non-OPTs progressed over time, where radiographic progression was concomitantly associated with clinical progression. Fifty-two percent of patients had incidentally identified probable non-OPTs. Twenty-five percent of patients were symptomatic at initial diagnosis, all of whom harbored tumors that grew on subsequent scans and required tumor-directed therapy. There were no clinical differences between probable non-OPTs localized to the brainstem vs other locations with respect to age, sex, concurrent optic pathway glioma, symptomology, and treatment. The average time from diagnosis to stabilization or decrease in tumor size was 2.34 years (SD, 2.15 years). Nineteen biopsied lesions were all histopathologically confirmed as tumor. Six children (9%) had deep extensive tumors, who presented earlier (mean age at diagnosis, 3.88 years), required multiple treatments, and had a shorter mean progression-free survival (48 months).
conclusionsOver half of children with NF1 in this study developed probable non-OPTs, the majority of which were clinically and radiographically progressive. While brainstem and nonbrainstem gliomas share similar clinical features and natural history, deep extensive tumors comprise a distinct aggressive group of tumors that warrant close attention.
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