Malignant peripheral nerve sheath tumor of the nasal cavity and nasopharynx in a child

Rationale: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms with only a few reported cases affecting the nasal cavity, paranasal sinuses, and anterior skull base. Patient concerns: A 12-year-old girl with a mass in her nose was admitted to the Department of Otorhinolaryngology of Shenzhen Children's Hospital. She had a 4-month history of progressive, unilateral right nasal obstruction, unilateral mucopurulent rhinorrhea, foul nasal odor, snoring, hyposmia, occasional epistaxis, and no headache, no facial numbness, without eye swelling and vision loss. Diagnose: A computed tomography (CT) scan of the paranasal sinuses showed a mass (right inflammatory polyp and calcification) involving the nasal cavity, the right maxillary sinusitis, ethmoid sinusitis. There was no nasal septum, orbital, or skull base involvement. On the basis of pathological and immunohistological findings, the tumor was diagnosed as an MPNST with heterogeneous components (cartilage and bone) mesenchymal differentiation after the operation. Interventions: The girl was treated by surgery and chemotherapy. Outcomes: The postoperative course was uneventful. There was no recurrence observed during the 3-year follow-up. Lessons: The primary MPNST in the nasal cavity is rare, if nasal neoplasms do not respond well to vasoconstrictors and glucocorticoids in children, the possibility of a tumor should be considered. If new organisms grow rapidly with hemorrhagic necrosis, the possibility of a malignant tumor is greater.