ReviewChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2020
Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease.
Review in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 31 citations in OpenAlex.
- The public health burden of nervous system tumors in Anambra State, Nigeria necessitates strategic resource allocation.Scientific reports · 2026Article
- A case report of malignant transformation in neurofibromatosis type 1: pain and rapid growth as key indicators for early biopsy.Frontiers in oncology · 2026Article
- Cervical Intranodal Schwannoma and Its Malignant Transformation: A Case Report With Literature Review.Clinical case reports · 2026Article
- Therapeutic Targeting of BET Proteins in Sarcoma.Molecular cancer therapeutics · 2025Review
- Unraveling novel variants in the NF1 gene and investigating potential therapeutic strategies.Scientific reports · 2025Article
- Malignant Peripheral Nerve Sheath Tumor (MPNST) of the Uterine Cervix - A Systematic Literature Review and New Case Report with Possible New Therapeutic Approaches.Geburtshilfe und Frauenheilkunde · 2025Article
- A Rare Case of Hodgkin Lymphoma With Isolated Epitrochlear Lymphadenopathy. Our Experience and Main Differential Diagnosis.Case reports in medicine · 2025Article
- Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Targeted Muscle Reinnervation and Regenerative Peripheral Nerve Interfaces for Prophylactic Pain Control in Neurofibromatosis Type 1 Amputees.Plastic and reconstructive surgery. Global open · 2023Article
- Case Report of a Patient With Malignant Peripheral Nerve Sheath Tumor Treated With Wide Resection.Cureus · 2023Article
- The therapeutic potential of neurofibromin signaling pathways and binding partners.Communications biology · 2023Review
- Case report: First case of undifferentiated embryonal sarcoma of the liver in a child with neurofibromatosis type 1, treated by hepatic chemoperfusion with transcatheter arterial chemoembolization.Frontiers in oncology · 2022Article
- 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
4 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMalignant peripheral nerve sheath tumors (MPNSTs) are rare yet highly aggressive soft tissue sarcomas. Children with neurofibromatosis type 1 (NF1) have a 10% lifetime risk for development of MPNST. Prognosis remains poor and survival seems worse for NF1 patients.
methodsThis narrative review highlights current practices and pitfalls in the management of MPNST in pediatric NF1 patients.
resultsPreoperative diagnostics can be challenging, but PET scans have shown to be useful tools. More recently, functional MRI holds promise as well. Surgery remains the mainstay treatment for these patients, but careful planning is needed to minimize postoperative morbidity. Functional reconstructions can play a role in improving functional status. Radiotherapy can be administered to enhance local control in selected cases, but care should be taken to minimize radiation effects as well as reduce the risk of secondary malignancies. The exact role of chemotherapy has yet to be determined. Reports on the efficacy of chemotherapy vary as some report lower effects in NF1 populations. Promisingly, survival seems to ameliorate in the last few decades and response rates of chemotherapy may increase in NF1 populations when administering it as part of standard of care. However, in metastasized disease, response rates remain poor. New systemic therapies are therefore desperately warranted and multiple trials are currently investigating the role of drugs. Targeted drugs are nevertheless not yet included in first line treatment.
conclusionBoth research and clinical efforts benefit from multidisciplinary approaches with international collaborations in this rare malignancy.
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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 Socratic graph.