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.

journal_name

J Pediatr Surg

authors

Sesenna E,Magri AS,Corradi D,Ferri T,Ferri A

doi

10.1016/j.jpedsurg.2011.04.058

subject

Has Abstract

pub_date

2011-08-01 00:00:00

pages

e9-12

issue

8

eissn

0022-3468

issn

1531-5037

pii

S0022-3468(11)00389-7

journal_volume

46

pub_type

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