ArticleJournal of cancer research and clinical oncology2010
Molecular evolution of a neurofibroma to malignant peripheral nerve sheath tumor (MPNST) in an NF1 patient: correlation between histopathological, clinical and molecular findings.
Article in Journal of cancer research and clinical oncology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 64 citations in OpenAlex.
- A Bayesian approach for diagnostic accuracy of malignant peripheral nerve sheath tumors: a systematic review and meta-analysis.Neuro-oncology · 2021Pooled it
- Treatment of Plexiform Neurofibromas : Current Perspectives on Surgery and Medical Treatment.Journal of Korean Neurosurgical Society · 2025Article
- A Sequencing Overview of Malignant Peripheral Nerve Sheath Tumors: Findings and Implications for Treatment.Cancers · 2025Review
- Review
- Interferon-Induced Transmembrane Protein 1 (IFITM1) Is Downregulated in Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors.International journal of molecular sciences · 2024Article
- Association of plexiform and diffuse neurofibromas with malignant peripheral nerve sheath tumor in NF I patients: a whole-body MRI assessment.Skeletal radiology · 2024Article
- Perinatal folate levels do not influence tumor latency or multiplicity in a model of NF1 associated plexiform-like neurofibromas.BMC research notes · 2023Article
- Establishment of in-hospital clinical network for patients with neurofibromatosis type 1 in Nagoya University Hospital.Scientific reports · 2021Article
- Limitations and benefits of FDG-PET/CT in NF1 patients with nerve sheath tumors: A cross-sectional/longitudinal study.Cancer science · 2021Observational
- Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1.Journal of neurosurgery · 2020Article
- Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
- Genetics of human malignant peripheral nerve sheath tumors.Neuro-oncology advances · 2020Review
- Genomic Status ofCancer research · 2018Article
- Telomere erosion in NF1 tumorigenesis.Oncotarget · 2017Article
- Article
- Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings.Human pathology · 2017Article
- Immortalization of human normal and NF1 neurofibroma Schwann cells.Laboratory investigation; a journal of technical methods and pathology · 2016Article
- Whole Exome Sequencing Reveals the Order of Genetic Changes during Malignant Transformation and Metastasis in a Single Patient with NF1-plexiform Neurofibroma.Clinical cancer research : an official journal of the American Association for Cancer Research · 2015Article
- A neurogenic tumor containing a low-grade malignant peripheral nerve sheath tumor (MPNST) component with loss of p16 expression and homozygous deletion of CDKN2A/p16: a case report showing progression from a neurofibroma to a high-grade MPNST.International journal of clinical and experimental pathology · 2015Article
- Wilms' tumor gene 1 (WT1) silencing inhibits proliferation of malignant peripheral nerve sheath tumor sNF96.2 cell line.PloS one · 2014Article
Corrections and comments
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Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
Abstract
objectiveNeurofibromatosis type 1 (NF1) patients have a 13% risk of developing a malignant peripheral nerve sheath tumor (MPNST). Many MPNSTs are histopathologically complex, with regions exhibiting features of the original benign plexiform neurofibroma (PNF), of an atypical PNF, or of MPNST showing varying degrees of de-differentiation. This study analyzed the genetic alterations associated with this pathological heterogeneity in order to identify the genetic processes involved in transformation from a benign to an aggressive malignant tumor.
methodsA histological and molecular analysis of a single MPNST tumor that was subdivided into three histopathologically distinct regions, a benign PNF (region 1), an atypical PNF (region 2), and a high-grade MPNST (region 3), was carried out. Tumor DNA from each region was analyzed in conjunction with the patient's lymphocyte DNA. Somatic mutation analyses included loss-of heterozygosity (LOH), MLPA analysis, NF1 gene sequencing, and a microarray comparative genomic hybridisation (array CGH) analysis.
resultsThe patient had a germline NF1 splice-site mutation. The NF1-associated LOH analysis found that LOH increased in the three tumor areas, with 9, 42, and 97% LOH evident in regions 1, 2, and 3, respectively. Additional genetic changes, including losses of TP53, RB1, CDKN2A, and of several oncogenes and cell-cycle genes, were found only in the malignant MPNST (region 3). Array CGH also identified genomic gains and losses in DNA from region 3. DISCUSSION: This is the first study that correlates the histological and molecular changes associated with MPNST development, confirming the significant cellular and genetic heterogeneity that poses both diagnostic and therapeutic challenges.
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