ArticleBrain pathology (Zurich, Switzerland)2009
Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases.
Article in Brain pathology (Zurich, Switzerland), 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 70 papers.
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Who cites it
70 citing papers in PubMed, 175 citations in OpenAlex.
- Glioblastoma With a Primitive Neuronal Component: A Case Report and Review of the Literature.Cureus · 2026Article
- How is rosette formation in brain tumours linked with cerebrospinal fluid spread?Brain tumor pathology · 2026Review
- Rare glioblastoma subtype masquerading as a poorly differentiated carcinoma: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Primary diffuse leptomeningeal glioblastoma: a case report and literature review.Journal of neuro-oncology · 2025Review
- IDH-mutant astrocytomas with primitive neuronal component have a distinct methylation profile and a higher risk of leptomeningeal spread.Acta neuropathologica · 2025Article
- Concurrent RB1 and P53 pathway disruption predisposes to the development of a primitive neuronal component in high-grade gliomas depending on MYC-driven EBF3 transcription.Acta neuropathologica · 2025Article
- Article
- Addressing barriers in diffuse intrinsic pontine glioma: the transformative role of lipid nanoparticulate drug delivery.ADMET & DMPK · 2024Review
- Mouse models of pediatric high-grade gliomas with MYCN amplification reveal intratumoral heterogeneity and lineage signatures.Nature communications · 2023Article
- Spinal Metastasis in a Patient with Supratentorial Glioblastoma with Primitive Neuronal Component: A Case Report with Clinical and Molecular Evaluation.Diagnostics (Basel, Switzerland) · 2023Article
- Review
- Freiburg Neuropathology Case Conference : A 58-year-old Patient Presenting with Dizziness, Nausea and Diplopia 12 Months after Total Resection of a Left-sided Temporal Glioblastoma Multiforme.Clinical neuroradiology · 2022Article
- Spectrum of paired-like homeobox 2b immunoexpression in pediatric brain tumors with embryonal morphology.Pathology international · 2022Article
- Molecular landscape of pediatric type IDH wildtype, H3 wildtype hemispheric glioblastomas.Laboratory investigation; a journal of technical methods and pathology · 2022Article
- Article
- Glioblastomas with primitive neuronal component harbor a distinct methylation and copy-number profile with inactivation of TP53, PTEN, and RB1.Acta neuropathologica · 2021Article
- A case series of extraneural metastatic glioblastoma at Memorial Sloan Kettering Cancer Center.Neuro-oncology practice · 2021Article
- A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components.Frontiers in oncology · 2021Article
- Cytology of Extraneural Metastases of Nonhematolymphoid Primary Central Nervous System Tumors: Six Cases with Histopathological Correlation and Literature Update.Acta cytologica · 2021Review
- A review of glioblastoma tumors with primitive neuronal component and a case report of a patient with this uncommon tumor at a rare location.Clinical case reports · 2020Article
10 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
13 authors at 9 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Central nervous system neoplasms with combined features of malignant glioma and primitive neuroectodermal tumor (MG-PNET) are rare, poorly characterized, and pose diagnostic as well as treatment dilemmas. We studied 53 MG-PNETs in patients from 12 to 80 years of age (median = 54 years). The PNET-like component consisted of sharply demarcated hypercellular nodules with evidence of neuronal differentiation. Anaplasia, as seen in medulloblastomas, was noted in 70%. Within the primitive element, N-myc or c-myc gene amplifications were seen in 43%. In contrast, glioma-associated alterations involved both components, 10q loss (50%) being most common. Therapy included radiation (78%), temozolomide (63%) and platinum-based chemotherapy (31%). Cerebrospinal fluid (CSF) dissemination developed in eight patients, with response to PNET-like therapy occurring in at least three. At last follow-up, 27 patients died, their median survival being 9.1 months. We conclude that the primitive component of the MG-PNET: (i) arises within a pre-existing MG, most often a secondary glioblastoma; (ii) may represent a metaplastic process or expansion of a tumor stem/progenitor cell clone; (iii) often shows histologic anaplasia and N-myc (or c-myc) amplification; (iv) has the capacity to seed the CSF; and (v) may respond to platinum-based chemotherapy regimens.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.