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The multimodality management of malignant peripheral nerve sheath tumours
Seres, Remus Hameed, Hassan McCabe, Martin G Russell, David Lee, Alexander T J
Seres, Remus
Hameed, Hassan
McCabe, Martin G
Russell, David
Lee, Alexander T J
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Abstract
Malignant peripheral nerve sheath tumours (MPNST) are aggressive sarcomas that have nerve sheath differentiation and can present at any anatomical site. They can arise from precursor neurofibroma in the context of neurofibromatosis type 1 (NF1) or as de novo and sporadic tumours in the absence of an underlying genetic predisposition. The primary therapeutic approach is most often radical surgery, with non-surgical modalities playing an important role, especially in locally advanced or metastatic cases. The aim of multimodality approaches is to optimize both local and systemic control while keeping to a minimum acute and late treatment morbidity. Advances in the understanding of the underlying biology of MPNSTs in both sporadic and NF-1-related contexts are essential for the management and implementation of novel therapeutic approaches.
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Date
2024
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Seres R, Hameed H, McCabe MG, Russell D, Lee ATJ. The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours. Cancers (Basel). 2024 Sep 26;16(19). PubMed PMID: 39409887. Pubmed Central PMCID: PMC11475700. Epub 2024/10/16. eng.