从良性神经纤维瘤到恶性周围神经鞘肿瘤(MPNST):多因素博弈。

IF 6.6 2区 医学 Q1 Medicine
Yanan Yu, Chengjiang Wei, Minghui Yue, Cheng Zhang, Yixiao Wang, Zhichao Wang
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引用次数: 0

摘要

几乎所有I型神经纤维瘤病(NF1)患者都会发展为良性周围神经肿瘤,称为神经纤维瘤,它来源于具有双等位基因NF1基因突变的神经嵴雪旺细胞谱系祖细胞。超过90%的NF1患者发展为真皮神经纤维瘤(DN), 25-50%发展为丛状神经纤维瘤(PN)。在8-13%的NF1患者中,PN可转化为恶性周围神经鞘肿瘤(MPNSTs),这是一种神经软组织肉瘤,是NF1患者死亡的主要原因。除了由良性神经纤维瘤(50%)引起外,MPNSTs也可自发发生(~40%)或放射治疗后发生(~10%)。MPNST的治疗仅限于完全切除阴性切缘。尽管如此,MPNST的高复发率仍然是一个主要问题。然而,完全切除癌前病变可大大降低患者的复发率和死亡率。因此,早期诊断和区分恶性、良性和癌前病变尤为重要。在良性神经纤维瘤向恶性发展的过程中,肿瘤形态、基因突变、多种信号通路相关蛋白表达、基因组不稳定等变化逐渐发生。在这篇综述中,我们详细介绍了这些变化,目的是确定恶性肿瘤开始的组织学和/或分子征象,以及最佳的治疗干预窗口,以抑制肿瘤进展并降低死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
From benign neurofibromas to malignant peripheral nerve sheath tumors (MPNST): a gaming among multiple factors.

Almost all patients of Neurofibromatosis Type I (NF1) develop benign peripheral nerve tumors called neurofibromas, which are derived from neural crest Schwann cell lineage progenitors with biallelic NF1 gene mutations. More than 90% of NF1 patients develop dermal neurofibromas (DN), and 25-50% develop plexiform neurofibromas (PN). In 8-13% of individuals with NF1, PN can transform into malignant peripheral nerve sheath tumors (MPNSTs), a type of nerve soft tissue sarcoma that is the main cause of mortality of NF1 patients. In addition to arising from benign neurofibromas (50%), MPNSTs can also occur spontaneously (~40%) or following radiation therapy (~10%). Treatment for MPNST is limited to complete resection with negative margins. Still, the high recurrence of MPNST is a major concern. However, full resection of the pre-malignant lesions can largely reduce the recurrence and mortality of patients. So, early diagnosis and distinguishing malignancy from benign and premalignant lesions are particularly important. During the progression from benign neurofibromas to malignancy, a variety of changes including tumor morphology, genetic mutations, expression of multiple signaling pathways-related proteins and genome instability gradually occur. In this review, we detail these changes with the goals of identifying the histological and/or molecular signs of malignancy initiation, and an optimal therapeutic intervention window, to inhibit tumor progression and reduce the rate of mortality.

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来源期刊
Cellular Oncology
Cellular Oncology Biochemistry, Genetics and Molecular Biology-Cancer Research
CiteScore
10.40
自引率
1.50%
发文量
0
审稿时长
16 weeks
期刊介绍: The Official Journal of the International Society for Cellular Oncology Focuses on translational research Addresses the conversion of cell biology to clinical applications Cellular Oncology publishes scientific contributions from various biomedical and clinical disciplines involved in basic and translational cancer research on the cell and tissue level, technical and bioinformatics developments in this area, and clinical applications. This includes a variety of fields like genome technology, micro-arrays and other high-throughput techniques, genomic instability, SNP, DNA methylation, signaling pathways, DNA organization, (sub)microscopic imaging, proteomics, bioinformatics, functional effects of genomics, drug design and development, molecular diagnostics and targeted cancer therapies, genotype-phenotype interactions. A major goal is to translate the latest developments in these fields from the research laboratory into routine patient management. To this end Cellular Oncology forms a platform of scientific information exchange between molecular biologists and geneticists, technical developers, pathologists, (medical) oncologists and other clinicians involved in the management of cancer patients. In vitro studies are preferentially supported by validations in tumor tissue with clinicopathological associations.
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