Malignant peripheral nerve sheath tumours (MPNSTs) are tumours that arise from or differentiate towards cells of the peripheral nerve sheath. They are extremely rare with an incidence of 0.001% in the general population. The most common association is with neurofibromatosis 1—an incidence of 5%–42% in this subset of the population is quoted in the literature. These tumours are aggressive with a high rate of local recurrence. Complete surgical excision remains the mainstay of treatment. A literature search found only three case reports of MPNSTs originating in the salivary glands—in all other cases the parotid gland. We present here the first documented case of a patient presented with an MPNST of the submandibular gland managed surgically by a specialty centre.
- cancer intervention
- nose and throat
- head and neck cancer
- oral and maxillofacial surgery
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Contributors MA wrote the paper and revised it based on feedback from his co-authors. RP helped write the results section and gave feedback on the final form. RS helped write the learning points and gave feedback on the final form. JA was lead consultant in the case. He highlighted the case as worth writing, explained the surgical technique and gave feedback on the final form. All contributors were involved in the patients care.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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