Malignant peripheral nerve sheath tumour (malignant epithelioid Schwannoma) of the parotid gland.

Problems/objectives: Malignant peripheral nerve sheath tumours are uncommon soft tissue tumours originating from Schwann cells or nerve sheath cells. Malignant epithelioid schwannoma is an aggressive variant of malignant peripheral nerve sheath tumour, and unfortunately is related with a high rate of recurrence and poor prognosis.

Methodology: In this study we present a rare case of malignant epithelioid schwannoma of the parotid gland and we discuss its origin, unusual presentation, and possible treatments options.

Results: Patient underwent total parotidectomy without neck dissection. Postoperative radiotherapy and chemotherapy management were not necessary.

Conclusion: Patients with malignant epithelioid schwannomas typically present with pain and/or rapid enlargement of a pre-existing lesion but the diagnosis of these tumours remains difficult as it is based primarily on clinical suspicion. There is no recognized management for this tumour yet while the prognosis seems to correlate best with the extent of surgical resection (Fig. 4, Ref. 16).