Publication year: 2012
Source:World Neurosurgery
Jason Voorhies, Eyas M. Hattab, Aaron A. Cohen-Gadol
Objective Malignant peripheral nerve sheath tumors are rare, and intracranial occurrences are even more rare. Treatment strategies have varied widely. The authors report the first case of a malignant peripheral nerve sheath tumor of the abducens nerve and provide a literature review that includes 61 cases of intracranial malignant peripheral nerve sheath tumors. Data were analyzed based on age, sex, treatment, survival, immunohistochemical staining, location, tumor grade, and neurofibromatosis association. These tumors more often affect males and the patients affected have a mean age of 39 years. Most patients underwent subtotal or gross total resection and radiation. Intracranial location varied, but most tumors occurred in the cerebellopontine angle. The literature demonstrates that intracranial malignant peripheral nerve sheath tumors are a heterogeneous group. Their association with neurofibromatosis (NF) is less clear than their extracranial counterparts. Prognosis is poor, but surgical resection and radiation can enhance chances for longer survival. Our aim was to elucidate information about these tumors. Clinical Presentation A 30-year-old woman presented with a 6-month history of headaches and diplopia. Results Neurologic examination revealed no focal deficit. Imaging revealed a heterogeneously enhancing retroclival mass without bony destruction. The tumor originated from the ipsilateral abducens nerve and extended into Dorello's canal; it adhered to the brainstem and cranial nerves. Pathology was consistent with low-grade malignant peripheral nerve sheath tumor. Conclusion At 3-month followup, the patient walked with a cane with no further deficits except mild ataxia which resolved within 6 months. MRI revealed a small residual tumor adherent to basilar artery. At 1-year followup and following ocular surgery to correct lateral rectus palsy, the patient was neurologically intact.
Source:World Neurosurgery
Jason Voorhies, Eyas M. Hattab, Aaron A. Cohen-Gadol
Objective Malignant peripheral nerve sheath tumors are rare, and intracranial occurrences are even more rare. Treatment strategies have varied widely. The authors report the first case of a malignant peripheral nerve sheath tumor of the abducens nerve and provide a literature review that includes 61 cases of intracranial malignant peripheral nerve sheath tumors. Data were analyzed based on age, sex, treatment, survival, immunohistochemical staining, location, tumor grade, and neurofibromatosis association. These tumors more often affect males and the patients affected have a mean age of 39 years. Most patients underwent subtotal or gross total resection and radiation. Intracranial location varied, but most tumors occurred in the cerebellopontine angle. The literature demonstrates that intracranial malignant peripheral nerve sheath tumors are a heterogeneous group. Their association with neurofibromatosis (NF) is less clear than their extracranial counterparts. Prognosis is poor, but surgical resection and radiation can enhance chances for longer survival. Our aim was to elucidate information about these tumors. Clinical Presentation A 30-year-old woman presented with a 6-month history of headaches and diplopia. Results Neurologic examination revealed no focal deficit. Imaging revealed a heterogeneously enhancing retroclival mass without bony destruction. The tumor originated from the ipsilateral abducens nerve and extended into Dorello's canal; it adhered to the brainstem and cranial nerves. Pathology was consistent with low-grade malignant peripheral nerve sheath tumor. Conclusion At 3-month followup, the patient walked with a cane with no further deficits except mild ataxia which resolved within 6 months. MRI revealed a small residual tumor adherent to basilar artery. At 1-year followup and following ocular surgery to correct lateral rectus palsy, the patient was neurologically intact.
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