The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours.

Autor: Seres, Remus, Hameed, Hassan, McCabe, Martin G., Russell, David, Lee, Alexander T. J.
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Zdroj: Cancers; Oct2024, Vol. 16 Issue 19, p3266, 14p
Abstrakt: Simple Summary: The landscape of malignant peripheral nerve sheath tumours (MPNSTs) is usually challenging both in terms of recognition and management. Despite a low incidence in the general population (0.001%), MPNST is an important cause of mortality in the neurofibromatosis type 1 (NF1) population. It is essential for a multi-disciplinary collaboration to achieve the best possible outcome. The aim of our paper was to contribute with a comprehensive review from the literature of the best multi-modality ways that show improvements in terms of survival and address potential future treatment approaches based on the molecular alterations seen in these tumours. 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. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index
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