Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumor in the Nasal Cavity.
intracranial extension
malignant peripheral nerve sheath tumor
nasal cavity
neurofibromatosis type 1
otolaryngology
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Sep 2023
Sep 2023
Historique:
accepted:
13
09
2023
medline:
16
10
2023
pubmed:
16
10
2023
entrez:
16
10
2023
Statut:
epublish
Résumé
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas, with 50% of cases associated with type 1 neurofibromatosis (NF-1). A 27-year-old male patient was referred to our department with an extended right nasal cavity MPNST. The lesion extended to the skull base, intracranial, parapharyngeal space, and infratemporal fossa. NF-1 was also confirmed by a neurologist. The patient was negative for distant metastases. Due to the tumor's proximity to vital structures, it was decided to treat it with chemotherapy and radiotherapy. Nasal cavity MPNSTs are particularly uncommon, with few reported cases. They should be included in the differential diagnosis of nasal masses or recurrent nosebleeds, particularly in patients with NF-1. Careful follow-up is essential to detect early recurrence, which contributes to a better prognosis.
Identifiants
pubmed: 37842475
doi: 10.7759/cureus.45175
pmc: PMC10575678
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e45175Informations de copyright
Copyright © 2023, Marini et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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