Mrugala Maciej M, Batchelor Tracy T, Plotkin Scott R
Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.
Curr Opin Neurol. 2005 Oct;18(5):604-10. doi: 10.1097/01.wco.0000179507.51647.02.
The intention of the authors is to provide the reader with an overview of the recent advances in the diagnosis and treatment of nerve sheath tumors. Vestibular schwannomas, neurogenetic syndromes such as schwannomatosis and multiple isolated neurofibromas, and malignant peripheral nerve sheath tumors are covered in this review.
Over the last year, literature focusing on different management strategies for patients with vestibular schwannomas dominated the field. Surgical options for this group of patients are changing. Stereotactic radiation is also employed more frequently with promising results. New insights into the biology of peripheral nerve tumor development and growth, including expression of vascular endothelial growth factor by vestibular schwannomas and the role of Notch signaling in malignant transformation of benign neurofibromas have been described. Diagnostic criteria for schwannomatosis, a recently described condition, are being developed. Several cases of multiple isolated neurofibromas and spinal neurofibromas were reported.
Peripheral nerve tumors are classified according to the specific features of cellular differentiation. The most common types include schwannoma and neurofibroma. These tumors can occur sporadically or as manifestations of genetic syndromes such as neurofibromatosis types 1 and 2 or schwannomatosis. The majority of peripheral nerve tumors are benign but malignant transformation does occur. Metastatic tumors can also affect peripheral nerves. The diagnostic modality of choice is magnetic resonance imaging. Positron emission tomography is a useful technique in the presurgical differentiation between benign and malignant peripheral nerve sheath tumors. Treatment is directed towards symptomatic control. Surgery, radiation and, in rare instances, chemotherapy are the major treatment modalities employed.
作者旨在为读者提供神经鞘瘤诊断与治疗的最新进展概述。本综述涵盖前庭神经鞘瘤、神经纤维瘤病和多发孤立性神经纤维瘤等神经遗传综合征以及恶性外周神经鞘瘤。
去年，专注于前庭神经鞘瘤患者不同管理策略的文献主导了该领域。这类患者的手术选择正在发生变化。立体定向放射治疗的应用也更为频繁，且效果良好。外周神经肿瘤发生和生长生物学方面有了新见解，包括前庭神经鞘瘤中血管内皮生长因子的表达以及Notch信号在良性神经纤维瘤恶变中的作用。神经纤维瘤病（一种最近描述的疾病）的诊断标准正在制定中。有多例多发孤立性神经纤维瘤和脊柱神经纤维瘤的病例报告。
外周神经肿瘤根据细胞分化的特定特征进行分类。最常见的类型包括神经鞘瘤和神经纤维瘤。这些肿瘤可散发性发生，或作为1型和2型神经纤维瘤病或神经纤维瘤病等遗传综合征的表现。大多数外周神经肿瘤是良性的，但确实会发生恶变。转移性肿瘤也可累及外周神经。首选的诊断方式是磁共振成像。正电子发射断层扫描是术前鉴别良性和恶性外周神经鞘瘤的有用技术。治疗旨在控制症状。手术、放疗以及在罕见情况下的化疗是主要的治疗方式。
