title: Malignant nerve sheath tumor involving glossopharyngeal, vagus and spinal nerve with intracranial-extracranial extension and systemic metastases in a patient with type 1 neurofibromatosis: A case report.
abstract: BACKGROUND
  Intracranial malignant peripheral nerve sheath tumors are an extremely rare pathology with a high morbidity and mortality. Epidemiological, clinical and prognostic data are scarce and with little certainty in the literature. The aim of this paper is to report for first time in English literature, the case of a patient with type 1 neurofibromatosis, who presented a malignant peripheral nerve sheath tumor that involved the left glossopharyngeal, vagus and spinal nerves with intracranial and extracranial extension through jugular foramen and systemic metastases.
  
  
  METHODS
  A 37 years-old female patient with malnutrition and Villaret́s syndrome. It was confirmed by brain magnetic resonance imaging and PET-CT the presence of a neoplasic lesion which was radiologically compatible with malignant peripheral nerve sheath tumor with systemic metastases. Partial surgical resection was performed; the patient postoperative course was without significant clinical improvement but with added peripheral facial palsy. The patient did not accept adjuvant management because of personal reasons.
  
  
  CONCLUSIONS
  Behavior therapy is unclear due to the low frequency of the disease and the lack of case series, representing a challenge for the physician in its approach and a poor prognosis for the patient.
authors.affiliation: Neurosurgery, National Autonomous University of Mexico (UNAM), Mexico City, Mexico; Neurosurgery Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico. Electronic address: drjrgm@hotmail.com.
authors.forename: José Raúl
authors.initials: JR
authors.lastname: Guerra-Mora
authors.affiliation: Neurosurgery, National Autonomous University of Mexico (UNAM), Mexico City, Mexico; Neurosurgery Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: Juan D
authors.initials: JD
authors.lastname: Del Castillo-Calcáneo
authors.affiliation: Neurosurgery, National Autonomous University of Mexico (UNAM), Mexico City, Mexico; Neurosurgery Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: María Elena
authors.initials: ME
authors.lastname: Córdoba-Mosqueda
authors.affiliation: Neurosurgery, National Autonomous University of Mexico (UNAM), Mexico City, Mexico; Neurosurgery Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: Jorge
authors.initials: J
authors.lastname: Yáñez-Castro
authors.affiliation: Neurosurgery, National Autonomous University of Mexico (UNAM), Mexico City, Mexico; Neurosurgery Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: Ulises
authors.initials: U
authors.lastname: García-González
authors.affiliation: Mexican School of Medicine, La Salle University. Mexico City, Mexico.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Soriano-Navarro
authors.affiliation: Pathology Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: Leticia
authors.initials: L
authors.lastname: Llamas-Ceras
authors.affiliation: Pathology Department, Mexican Oil Company (Pemex) High Specialty Hospital, Mexico City, Mexico.
authors.forename: Rosa María
authors.initials: RM
authors.lastname: Vicuña-González
keywords: Glossopharyngeal; Malignant peripheral nerve sheath tumor; Neurofibromatosis; Spinal; Vagus; Villaret’s syndrome