文章：

恶性外周神经鞘瘤的多模式综合治疗

The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours

原文发布日期：26 September 2024

DOI: 10.3390/cancers16193266

类型: Article

开放获取: 是

英文摘要：

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.

摘要翻译： 

恶性外周神经鞘瘤（MPNST）是具有神经鞘分化的侵袭性肉瘤，可发生于任何解剖部位。其既可源于1型神经纤维瘤病（NF1）背景下的前体神经纤维瘤，也可在没有潜在遗传易感性的情况下作为原发散发性肿瘤出现。主要治疗手段通常为根治性手术，非手术疗法亦发挥重要作用，尤其在局部晚期或转移性病例中。多学科综合治疗的目标是在最大限度降低急性及远期治疗相关并发症的同时，实现对局部和全身病情的最优控制。深入理解散发性及NF1相关性MPNST的生物学机制，对于疾病管理和新型治疗策略的实施具有至关重要的意义。

原文链接：

The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours