Seres Remus, Hameed Hassan, McCabe Martin G, Russell David, Lee Alexander T J
Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester M20 4BX, UK.
Division of Cancer Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester M13 9PL, UK.
Cancers (Basel). 2024 Sep 26;16(19):3266. doi: 10.3390/cancers16193266.
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.
恶性外周神经鞘瘤（MPNST）是具有神经鞘分化的侵袭性肉瘤，可出现在任何解剖部位。它们可源于1型神经纤维瘤病（NF1）背景下的前驱神经纤维瘤，或在无潜在遗传易感性的情况下作为新发和散发性肿瘤出现。主要治疗方法通常是根治性手术，非手术方式也发挥着重要作用，尤其是在局部晚期或转移性病例中。多模式治疗方法的目的是在将急性和晚期治疗并发症降至最低的同时，优化局部和全身控制。了解散发性和NF-1相关背景下MPNST的潜在生物学进展对于新型治疗方法的管理和实施至关重要。
