ArticleActa bio-medica : Atenei Parmensis2020
Advanced pharmacological therapies for neurofibromatosis type 1-related tumors.
Article in Acta bio-medica : Atenei Parmensis, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 17 citations in OpenAlex.
- The Complexities of Periorbital Neurofibroma: Diagnostic Ambiguity and Therapeutic Dilemmas: A Case Report and Literature Review.Diagnostics (Basel, Switzerland) · 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
- Outcome of Surgical Intervention in Giant Neurofibromas in Two Sisters with Neurofibromatosis Type 1: A Case Series and Literature Review.Clinical, cosmetic and investigational dermatology · 2026Article
- Clinical characteristics and healthcare burden of neurofibromatosis type 1 in Saudi Arabia: a single centre experience.Hereditary cancer in clinical practice · 2025Article
- Neurofibromatosis Type 1 and the Search for Effective Tumor Therapies Using High-Throughput Drug Screening.Current oncology (Toronto, Ont.) · 2025Review
- Neurofibromatosis type 1 adult surveillance form for Austria.Wiener klinische Wochenschrift · 2025Article
- [A case report of giant neurofibromatosis of maxillofacial, neck and chest was treated by multidisciplinary cooperation].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2025Article
- Adeno-associated viral vector targeted evolution for neurofibromatosis gene delivery.Trends in molecular medicine · 2025Review
- Article
- [Progress in neurosurgical treatment of neurofibromatosis type 1].Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery · 2024Review
- Neurofibromatosis Type 1-Retinal Alterations Detectable with Optical Coherence Tomography Angiography.Diagnostics (Basel, Switzerland) · 2024Article
- Maintaining Engagement in Adults with Neurofibromatosis Type 1 to Use the iCanCope Mobile Application (iCanCope-NF).Cancers · 2023Article
- Treatment decisions and the use of MEK inhibitors for children with neurofibromatosis type 1-related plexiform neurofibromas.BMC cancer · 2023Review
- Neurofibromatosis type 1 system-based manifestations and treatments: a review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Review
- Pediatric traumatic brain injury: a new relation between outcome and neutrophil-to-lymphocite ratio.Acta bio-medica : Atenei Parmensis · 2022Observational
- Therapeutic aspects of Sydenham's Chorea: an update.Acta bio-medica : Atenei Parmensis · 2022Review
- Human Herpes Virus 7-related encephalopathy in children.Acta bio-medica : Atenei Parmensis · 2022Article
- Neurofibromatosis in Children: Actually and Perspectives.Children (Basel, Switzerland) · 2022Review
- Neurofibromatosis in the Era of Precision Medicine: Development of MEK Inhibitors and Recent Successes with Selumetinib.Current oncology reports · 2021Review
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Neurofibromatosis Type 1 (NF1) is an autosomal dominant tumor-predisposition disorder that is caused by a heterozygous loss of function variant in the NF1 gene, which encodes a protein called neurofibromin. The absence of neurofibromin causes increased activity in the Rat sarcoma protein (RAS) signalling pathway, which results in an increased growth and cell proliferation. As a result, both oncological and non-oncological comorbidities contribute to a high morbidity and mortality in these patients. Optic pathways gliomas, plexiform neurofibromas and malignant peripheral nerve sheath tumor (MPNST) are the most frequent NF1-associated tumors. The treatment of these complications is often challenging, since surgery may not be feasible due to the location, size, and infiltrative nature of these tumors, and standard chemotherapy or radiotherapy are burdened by significant toxicity and risk for secondary malignancies. For these reasons, following the novel discoveries of the pathophysiological mechanisms that lead to cell proliferation and tumorigenesis in NF1 patients, emerging drugs targeting specific signalling pathways (i.e. the MEK/ERK cascade), have been developed with promising results.
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