title: Long-term survival with an intracerebral progressive nerve sheath tumour.
abstract: BACKGROUND
  Malignant peripheral nerve sheath tumours (MPNSTs) are rare neoplasias, often having progressive growth and an unfavourable outcome. Patients relapse regularly and the tumour metastasizes frequently. Early diagnosis and intense therapy, including radical resection and adjuvant radio- and chemotherapy, are necessary to achieve cure or prolonged survival.
  
  
  METHODS
  A woman aged 60 years, survived 137 months from an initial diagnosis of fibroma of the sinus, which later proved to be intracerebral progressive malignant metastasized nerve sheath tumour.
  
  
  CONCLUSIONS
  A literature review and various case reports showed that MPNSTs are generally highly malignant, and often cannot be cured. Patients can be cured if the tumour is completely removed following adjuvant radio- and chemotherapy, depending on the tumour location.
authors.affiliation: Dept. of General ENT, ENT University Hospital, Medical University of Graz, Austria. bernd.hoeller@klinikum-graz.at
authors.forename: B
authors.initials: B
authors.lastname: Hoeller
authors.affiliation: Dept. of General ENT, ENT University Hospital, Medical University of Graz, Austria.
authors.forename: W
authors.initials: W
authors.lastname: Koele
mesh_terms: D044963:Biopsy, Fine-Needle; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000628:therapy; D049268:Positron-Emission Tomography; D013997:Time Factors; D014057:Tomography, X-Ray Computed