A well 74 -year-old woman presented to routine ophthalmology clinic instituted following treatment of a frontoethmoidal sarcoma initially excised in 1989 and diagnosed then as a sinus mucosal melanoma. At review in ophthalmology clinic, a reduction in right visual fields was noted. CT scan showed recurrence of a mass now involving the frontoethmoidal region, frontal sinus and abutting the cribiform plate. Endoscopic biopsy confirmed the recurrence as a low-grade biphenotypic sarcoma. This was discussed at the sarcoma multidisciplinary meeting. Using a three-dimensional printed model of the patients skull for planning, primary surgery with craniofacial resection combining intracranial and transfacial approaches with reconstruction was decided on. The implications of no treatment would be tumour involvement of the dura and brain as well as the right only-seeing eye. Craniofacial surgery would involve otolaryngolical, neurosurgical and maxillofacial multispecialty involvement and close teamwork. The goal was en bloc excision with negative surgical margins.
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.
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