title: Malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem:  multimodality treatment with survival of 27 months. A case report and review of the literature.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurosurgery, St John Providence Hospital and Medical Centers, Michigan State University, Southfield, Michigan 48075, USA. kristophe.karami@stjohn.org
authors.forename: Kristophe J
authors.initials: KJ
authors.lastname: Karami
authors.forename: Prashant S
authors.initials: PS
authors.lastname: Kelkar
authors.forename: Michael P
authors.initials: MP
authors.lastname: Verdon
authors.forename: Inga S
authors.initials: IS
authors.lastname: Grills
authors.forename: Dennis I
authors.initials: DI
authors.lastname: Bojrab
authors.forename: Daniel R
authors.initials: DR
authors.lastname: Pieper
mesh_terms: D001933:Brain Stem / Q000473:pathology* / Q000601:surgery; D003131:Combined Modality Therapy / Q000379:methods; D003390:Cranial Nerve Neoplasms / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery / Q000628:therapy; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D000160:Vestibulocochlear Nerve Diseases / Q000473:pathology* / Q000601:surgery / Q000628:therapy*; D055815:Young Adult