Malignant Peripheral Nerve Sheath Tumor of the Vestibulocochlear Nerve and Brainstem: Multimodality

BACKGROUND AND IMPORTANCE: Malignant peripheral nerve sheath tumors are the most common malignant mesenchymal tumors of soft tissues, but they are very rare when found to arise from a cranial nerve and when not in association with neurofibromatosis. These tumors are highly malignant and carry a poor prognosis with survival usually less than 6 months. CLINICAL PRESENTATION: The authors report the case of a 23-year-old female with no history of phakomatoses, previous irradiation, or known genetic disorders, who presented with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem. Multiple staged skull base approaches were carried out with maximal possible resection. Adjunctive therapies including standard radiation therapy, intensity-modulated radiation therapy, and stereotactic gamma knife radiosurgery were used with an ultimate patient survival of 27 months. CONCLUSION: To our knowledge, this is the first report describing a patient with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem treated with staged surgical approaches in conjunction with multiple forms of radiotherapy and having a significant survival of more than 2 years. ABBREVIATIONS: CN: cranial nerve MPNST: malignant peripheral nerve sheath tumor NF: neurofibromatosis SRS: stereotactic radiosurgery

http://journals.lww.com/neurosurgery/Fulltext/2011/11000/Malignant_Peripheral_Nerve_Sheath_Tumor_of_the.17.aspx

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Júlio Leonardo B. Pereira
http://lattes.cnpq.br/7687651239699170
http://www.neurocirurgiabr.comhttp://www.radiocirurgia.org

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