Malignant peripheral nerve sheath tumour arising in the submandibular gland

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.