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Curr Opin Neurol. 2005 Oct;18(5):604-10.

Peripheral and cranial nerve sheath tumors.

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  • 1Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.



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.

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