NF2-related Schwannomatosis (NF2-SWN) remains a disorder with few effective treatment options. Patients develop vestibular schwannomas (VSs) on both auditory nerves, which gradually impair hearing and often result in significant communication difficulties, social withdrawal, and higher rates of depression. Progress in understandingNF2-SWN biology and translating discoveries into therapies has been slowed by the absence of robust animal models that faithfully reproduce both tumor behavior and the associated neurological deficits. In this review, we summarized the development of animal models that not only reproduce tumor growth in the peripheral nerve microenvironment but also reproduce tumor-induced neurological symptoms, such as hearing loss and ataxia. We further highlight the currently available organotypic models forNF2-SWN. Together, these systems provide an essential foundation for advancing mechanistic studies and accelerating the development of effective therapies for this devastating disorder.
NF2相关神经鞘瘤病（NF2-SWN）目前仍是一种有效治疗方案有限的疾病。患者双侧听神经上生长前庭神经鞘瘤（VSs），这些肿瘤逐渐损害听力，常导致严重的交流障碍、社交回避及更高的抑郁发生率。由于缺乏能够忠实模拟肿瘤行为及相关神经功能缺损的可靠动物模型，对NF2-SWN生物学机制的理解以及相关疗法转化的研究进展受到限制。本综述总结了动物模型的发展进展，这些模型不仅能重现外周神经微环境中的肿瘤生长，还能模拟肿瘤引起的神经症状，如听力丧失和共济失调。我们进一步重点介绍了目前可用于NF2-SWN研究的类器官模型。这些系统共同为推进机制研究和加速针对这一破坏性疾病的有效疗法开发提供了重要基础。
Advancements in Preclinical Models forNF2-Related Schwannomatosis Research
肿瘤(癌症)患者之家