ReviewMolecular cancer research : MCR2019
Malignant Peripheral Nerve Sheath Tumors: From Epigenome to Bedside.
Review in Molecular cancer research : MCR, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
44 citing papers in PubMed, 1 synthesis or guideline pooled it, 83 citations in OpenAlex.
- Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis.World journal of surgical oncology · 2020Pooled it
- Diagnosing metastatic angiosarcoma arising from MPNST using H3K27me3 staining in NF1: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Clinical trial landscape of malignant peripheral nerve sheath tumors: challenges and opportunities.Frontiers of medicine · 2025Article
- From benign neurofibromas to malignant peripheral nerve sheath tumors (MPNST): a gaming among multiple factors.Cellular oncology (Dordrecht, Netherlands) · 2025Review
- Malignant Transformation of a Vestibular Schwannoma Without Previous Radiation Exposure: Illustrative Case and Literature Review.Journal of neurological surgery reports · 2025Article
- Schwannoma with uncharacteristic rapid growth and high Ki-67 index: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Galectin-1 Inhibition as a Strategy for Malignant Peripheral Nerve Sheath Tumor Treatment.Cells · 2025Article
- Schwannoma in the lower inner quadrant of the right breast in a male patient: A rare case report.Medicine · 2025Article
- CD274/PD-L1 copy number gained malignant peripheral nerve sheath tumor: A case report and literature review.Medicine · 2025Review
- Targeting the Galectin-1/Ras Interaction for Treating Malignant Peripheral Nerve Sheath Tumors.Research square · 2024Article
- Proof of Concept for Genome Profiling of the Neurofibroma/Sarcoma Sequence in Neurofibromatosis Type 1.International journal of molecular sciences · 2024Article
- Conditionally replicative adenovirus as a therapy for malignant peripheral nerve sheath tumors.Molecular therapy. Oncology · 2024Article
- Bulky malignant peripheral nerve sheath tumour of the left thigh in a pregnant woman presenting with a pathological fracture of the proximal femur.BMJ case reports · 2024Article
- Role of catecholamine synthases in the maintenance of cancer stem-like cells in malignant peripheral nerve sheath tumors.Cancer science · 2024Article
- Brain metastasis and intracranial leptomeningeal metastasis from malignant peripheral nerve sheath tumors: illustrative cases.Journal of neurosurgery. Case lessons · 2023Article
- Deregulated expression of polycomb repressive complex 2 target genes in a NF1 patient with microdeletion generating the RNF135-SUZ12 chimeric gene.Neurogenetics · 2023Article
- Pigmented malignant peripheral nerve sheath tumor of the nail unit arising in a patient with neurofibromatosis type I.JAAD case reports · 2023Article
- CT of malignant peripheral nerve sheath tumor.Radiology case reports · 2023Article
- HDAC inhibitors enhance the anti-tumor effect of immunotherapies in hepatocellular carcinoma.Frontiers in immunology · 2023Review
- Gain-of-Function Variomics and Multi-omics Network Biology for Precision Medicine.Methods in molecular biology (Clifton, N.J.) · 2023Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Malignant peripheral nerve sheath tumors (MPNST) are aggressive sarcomas typically developing in the context of neurofibromatosis type 1 (NF-1). With the exception of surgical resection, these tumors are resistant to all current therapies, and unresectable, recurrent, or metastatic tumors are considered incurable. Preclinical studies have identified several novel candidate molecular targets for therapeutic intervention, but, to date, targeted therapies have proven ineffective. Recent studies have identified recurrent mutations in polycomb repressive complex 2 (PRC2) core components, embryonic ectoderm development protein (EED) and suppressor of zeste 12 homolog (SUZ12), in MPNST. These mutations result in global loss of the histone H3 lysine 27 trimethylation epigenetic mark, normally deposited by PRC2, and subsequent gain in acetylation at this residue. This altered chromatin state has been shown to promote MPNST malignancy; however, acetylation at this residue sensitizes MPNSTs to BRD4 and bromodomain and extra-terminal domain inhibition. Interestingly, the catalytic component of PRC2, enhancer of zeste homolog 2 (EZH2), is not mutated in MPNST, hinting that a noncanonical, PRC2-independent function of EZH2 may play a role in this cancer. This review examines the pathobiology of MPNST, the contribution of PRC2 subunits to this process, and the prospects for PRC2-related therapies for this cancer. IMPLICATIONS: Identification of mutations in the PRC2 components EED and SUZ12 in the majority of MPNSTs may imply noncanonical oncogenic activities of the intact component, EZH2, and provide new opportunities for therapeutic intervention.
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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.