title: Malignant peripheral nerve sheath tumor of the vagus nerve in a teenager with the neurofibromatosis 1 gene mutation: a case report.
abstract: Malignant peripheral nerve sheath tumors account for approximately 5% to 10% of all soft tissue sarcomas in which 25% to 50% are diagnosed in patients with neurofibromatosis 1 (NF1). Tumors are often located in the proximal portion of the upper and lower extremities and trunk, whereas cervical vagus nerve localizations are extremely rare, and the English literature is limited to isolated case reports. Malignant peripheral nerve sheath tumors usually affect adults. However, earlier presentation is described in patients with the NF1 mutation. The authors describe a very rare case of malignant peripheral nerve sheath tumor of the vagus nerve in a teenage patient with NF1 focusing on surgical management of this uncommon pathology and its histopathologic features to underline the importance of differential diagnosis and early treatment of this rare and aggressive tumor.
authors.affiliation: Maxillo-Facial Surgery Division, Head and Neck Department, University Hospital of Parma, 43100 Parma, Italia.
authors.forename: Enrico
authors.initials: E
authors.lastname: Sesenna
authors.forename: Alice Sara
authors.initials: AS
authors.lastname: Magri
authors.forename: Domenico
authors.initials: D
authors.lastname: Corradi
authors.forename: Teore
authors.initials: T
authors.lastname: Ferri
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferri
mesh_terms: D000293:Adolescent; D001483:Base Sequence; D003390:Cranial Nerve Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005260:Female; D016514:Genes, Neurofibromatosis 1; D006801:Humans; D009333:Neck; D018317:Nerve Sheath Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000235:genetics; D017384:Sequence Deletion; D020421:Vagus Nerve Diseases / Q000150:complications* / Q000473:pathology / Q000601:surgery