Abstract

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.