Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas, often affecting the head and neck, with higher incidence in neurofibromatosis type 1. Early diagnosis is crucial to prevent recurrence and metastasis. We report a 78-year-old male with a rapidly enlarging right nasal cavity mass over 4 months, associated with recurrent epistaxis. His medical history included diabetes, hypertension, and tobacco use. Examination revealed a firm, non-adherent mass obstructing the nasal cavity. Computed tomography (CT) imaging localized it to the right inferior turbinate. Due to size and location, biopsy was not performed. Surgical resection confirmed a high-grade MPNST (FNCLCC grade 3). Chemotherapy was initiated postoperatively, but the patient passed away soon after, highlighting the tumor's aggressiveness. MPNSTs should be considered in nasal mass differentials, especially in elderly smokers. Early surgery and chemotherapy may improve outcomes, emphasizing the need for a multidisciplinary approach.

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Publication Details

Journal
Journal of Surgical Case Reports
Published
2025-04-29
DOI
https://doi.org/10.1093/jscr/rjaf349
Primary Topic
Neurofibromatosis and Schwannoma Cases
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article
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article

Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge

Malik Boulaadas, Bouchra Dani, Amine Oussalem, Anas Azgaoui
Journal of Surgical Case Reports
Neurofibromatosis and Schwannoma Cases
article

Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge

Malik Boulaadas, Bouchra Dani, Amine Oussalem, Anas Azgaoui
article en

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas, often affecting the head and neck, with higher incidence in neurofibromatosis type 1. Early diagnosis is crucial to prevent recurrence and metastasis. We report a 78-year-old male with a rapidly enlarging right nasal cavity mass over 4 months, associated with recurrent epistaxis. His medical history included diabetes, hypertension, and tobacco use. Examination revealed a firm, non-adherent mass obstructing the nasal cavity. Computed tomography (CT) imaging localized it to the right inferior turbinate. Due to size and location, biopsy was not performed. Surgical resection confirmed a high-grade MPNST (FNCLCC grade 3). Chemotherapy was initiated postoperatively, but the patient passed away soon after, highlighting the tumor's aggressiveness. MPNSTs should be considered in nasal mass differentials, especially in elderly smokers. Early surgery and chemotherapy may improve outcomes, emphasizing the need for a multidisciplinary approach.

Journal of Surgical Case ReportsVol. 2025(5)
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Neurofibromatosis and Schwannoma Cases
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Malignant peripheral nerve sheath tumor of the nasal cavity: a rare diagnostic and therapeutic challenge — Malik Boulaadas, Bouchra Dani, et al. · Journal of Surgical Case Reports (2025) | TGRS Research Map | TGRS