Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumor in the Nasal Cavity
Katerina Marini,
James Philip Skliris,
Konstantinos Garefis
et al.
Abstract: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 i… Show more
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