ReviewCellular oncology (Dordrecht, Netherlands)2025
From benign neurofibromas to malignant peripheral nerve sheath tumors (MPNST): a gaming among multiple factors.
Review in Cellular oncology (Dordrecht, Netherlands), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed.
- Neurofibromatosis Type 1 Tumor Involving the Anterosuperior Mediastinum With a Rare c.147C>G Germline Mutation: A Case Report.Clinical case reports · 2026Article
- Circulating Tumor DNA in Neurofibromatosis Type 1: Translating Molecular Discovery into Clinical Surveillance.Diagnostics (Basel, Switzerland) · 2026Review
- Peripheral nerve sheath tumors-on-a-chip: Next-generation platforms for mechanistic and therapeutic studies.Materials today. Advances · 2026Article
- Decreased PP2A expression and activity represent a therapeutic target for plexiform neurofibroma.Acta neuropathologica communications · 2026Article
- Lithium carbonate-loaded polymeric nano-micelles for enhanced antitumor activity against NF1-associated malignant peripheral nerve sheath tumorsNanoscale advances · 2026Article
- Genetic and non-genetic factors influencing phenotypic variability in neurofibromatosis type 1.Orphanet journal of rare diseases · 2026Review
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- Giant cervicothoracic malignant peripheral nerve sheath tumor in neurofibromatosis type I: a case report of integrated management with multimodal guidance.Frontiers in surgery · 2026Article
- Giant cervicothoracic malignant peripheral nerve sheath tumor in neurofibromatosis type 1: a case report of multidisciplinary management and literature review.Frontiers in oncology · 2026Article
- Achieving Breast Symmetry in a Patient With Neurofibromatosis-1: A Case Report and Literature Review.Plastic and reconstructive surgery. Global open · 2026Article
- Rectal malignant peripheral nerve sheath tumor with extremely high Ki-67 index (80%) and concomitant meningioma history: a case report.Frontiers in oncology · 2026Article
- Mesenteric neurofibromatosis complicated by acute appendicitis: case report and review of the literature.Frontiers in oncology · 2026Article
- Clinical trial landscape of malignant peripheral nerve sheath tumors: challenges and opportunities.Frontiers of medicine · 2025Article
- Clinical characteristics and healthcare burden of neurofibromatosis type 1 in Saudi Arabia: a single centre experience.Hereditary cancer in clinical practice · 2025Article
- Vitamin D Binding Protein, a Ligand of Integrin beta 1, Motivates Both Tumor Cells and Schwann Cells to Promote Perineural Invasion in Pancreatic Ductal Adenocarcinoma.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- Pathological Fracture in the Metatarsal Bone Due to an Intraosseous Schwannoma: A Case Report.Cureus · 2025Article
- Case Report: Malignant transformation of ganglioneuroma in the rectovaginal septum to malignant peripheral nerve sheath tumor based on neurofibromas.Frontiers in oncology · 2025Article
- Case Report: Malignant peripheral nerve sheath tumor of the thoracic spine with postoperative recurrence and intracranial metastasis-a review of the literature.Frontiers in oncology · 2025Article
Corrections and comments
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Authors and funding
6 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Almost all patients of Neurofibromatosis Type I (NF1) develop benign peripheral nerve tumors called neurofibromas, which are derived from neural crest Schwann cell lineage progenitors with biallelic NF1 gene mutations. More than 90% of NF1 patients develop dermal neurofibromas (DN), and 25-50% develop plexiform neurofibromas (PN). In 8-13% of individuals with NF1, PN can transform into malignant peripheral nerve sheath tumors (MPNSTs), a type of nerve soft tissue sarcoma that is the main cause of mortality of NF1 patients. In addition to arising from benign neurofibromas (50%), MPNSTs can also occur spontaneously (~40%) or following radiation therapy (~10%). Treatment for MPNST is limited to complete resection with negative margins. Still, the high recurrence of MPNST is a major concern. However, full resection of the pre-malignant lesions can largely reduce the recurrence and mortality of patients. So, early diagnosis and distinguishing malignancy from benign and premalignant lesions are particularly important. During the progression from benign neurofibromas to malignancy, a variety of changes including tumor morphology, genetic mutations, expression of multiple signaling pathways-related proteins and genome instability gradually occur. In this review, we detail these changes with the goals of identifying the histological and/or molecular signs of malignancy initiation, and an optimal therapeutic intervention window, to inhibit tumor progression and reduce the rate of mortality.
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