Na Brian, Haist Blake, Shah Shilp R, Sabiston Graeme, Jonas Steven J, Vitte Jeremie, Wirz Richard E, Giovannini Marco
Department of Head and Neck Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA 90095, USA.
UCLA Neuro-Oncology Program, Department of Neurology, UCLA David Geffen School of Medicine, Los Angeles, CA 90095, USA.
Biomedicines. 2024 Sep 2;12(9):1986. doi: 10.3390/biomedicines12091986.
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder resulting from mutations in the gene. Patients harboring these mutations are predisposed to a spectrum of peripheral nerve sheath tumors (PNSTs) originating from Schwann cells, of which malignant peripheral nerve sheath tumors (MPNSTs) are the deadliest, with limited treatment options. Therefore, an unmet need still exists for more effective therapies directed at these aggressive malignancies. Cold atmospheric plasma (CAP) is a reactive oxygen species (ROS) and reactive nitrogen species (RNS) generating ionized gas that has been proposed to be a potential therapeutic modality for cancer. In this study, we sought to determine the effects of CAP on NF1-associated PNSTs. Utilizing established mouse and human cell lines to interrogate the effects of CAP in both in vitro and in vivo settings, we found that NF1-associated PNSTs were highly sensitive to CAP exposure, resulting in cell death. To our knowledge, this is the first application of CAP to NF1-associated PNSTs and provides a unique opportunity to study the complex biology of NF1-associated tumors.
1型神经纤维瘤病（NF1）是一种常染色体显性疾病，由该基因的突变引起。携带这些突变的患者易患一系列源自施万细胞的周围神经鞘瘤（PNST），其中恶性周围神经鞘瘤（MPNST）最为致命，治疗选择有限。因此，针对这些侵袭性恶性肿瘤的更有效疗法仍存在未满足的需求。冷大气等离子体（CAP）是一种能产生活性氧（ROS）和活性氮（RNS）的电离气体，已被提议作为一种潜在的癌症治疗方式。在本研究中，我们试图确定CAP对NF1相关PNST的影响。利用已建立的小鼠和人类细胞系来研究CAP在体外和体内环境中的作用效果，我们发现NF1相关PNST对CAP暴露高度敏感，从而导致细胞死亡。据我们所知，这是CAP首次应用于NF1相关PNST，为研究NF1相关肿瘤的复杂生物学特性提供了独特的机会。
