P21.11 Malignant nerve sheath tumor involving glossopharyngeal vagus and spinal nerve with intracranial-extracranial extension and systemic spread in a patient with type 1 neurofibromatosis

摘要

Intracranial malignant peripheral nerve sheath tumors are extremely a rare disease with high morbidity and mortality. Epidemiological, clinical and prognostic data are scarce and little certainty in the literature. The aim of this paper is to report for the first time in English literature, the case of a patient with neurofibromatosis type 1, which 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.Female 37 years old with malnutrition and Villaret syndrome. It was confirmed by magnetic resonance imaging of brain and PET-CT, the presence of a tumor lesion compatible with malignant peripheral nerve sheath tumor with systemic dissemination. Partial resection of the lesion was performed, the patient postoperative course was without significant clinical improvement with added facial paresis. She did not accept adjuvant management for personal reasons.Behavior therapy is unclear due to the low frequency of the disease and the lack of case series, giving now a challenge for the physician in his approach and a poor prognosis to the patient.