Malignant Peripheral Nerve Sheath Tumor of the Parotid Gland

Abstract

Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, accounts for < 5% of all soft tissue sarcomas, and carries a poor prognosis. In this article, we report a case of MPNST of parotid gland in a 45-year-old male and review its diagnostic and therapeutic challenges. A 45-year-old male presented with right parotid swelling for 2 years with rapid increase in size over the last 3 months. He underwent right total conservative parotidectomy with selective neck dissection. Reconstruction was done with microvascular anterolateral thigh flap. On immunohistochemistry, the tumor cells expressed CD 56 diffusely and S 100 focally. Tumor was immunonegative for CK, Desmin, SOX -10, and SMA consistent with MPNST. The MPNSTs arising as parotid mass are very rare and aggressive tumors. The role of IHC is paramount in establishing the diagnosis. Multimodal management including wide surgical resection, neck dissection, and adjuvant chemoradiotherapy is the choice of treatment.

Not So Benign Bell’s Palsy: Malignant Peripheral Nerve Sheath Tumor of the Facial Nerve Involving the Temporal Bone

“…To detect local recurrence, MRI is recommended yearly for the first 5 years after resection. 16,24 Our patient fortunately did not have any distant metastasis based on 18 F-FDG PET at the time of diagnosis, 18 months after the onset of her symptoms, but any further delay in diagnosis and management most likely would have affected her morbidity and mortality significantly.…”

“…8,15,16 MPNST with involvement of the temporal bone commonly presents as enlarging, painful masses with intact facial nerve function, rather than facial palsy. 17,18 Biopsy is required for diagnosis, and imaging is needed for staging and treatment planning. 19 CT of the chest is usually used for staging given the high potential of metastatic spread to the lungs.…”

Case report of malignant peripheral nerve sheath tumor of parotid in a patient with neurofibromatosis type 1

“…Malignant tumors are outnumbered by benign mesenchymal tumors. 1 The ratio of benign to malignant lesions described in the literature is 2.4:1 to 18:1. The incidence of MPNST in the parotid gland is very rare, about 0.01%.…”