Malignant peripheral nerve sheath tumors of the lateral skull base

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive spindle cell tumors that develop from peripheral nerve sheath cells and account for 5% of all soft tissue sarcomas. MPNSTs involving the lateral skull base are an extremely rare subgroup of these lesions. The article deals with the case of a 51-year-old man with a huge primary MPNST: the disease involved the left frontozygomatic and retroauricolar regions of the scalp with erosion of the outer table of the calvaria and diffuse infiltration of the temporal, infratemporal, and pterygoid fossae. Radical surgery via left transfacial- transparotid approach and reconstruction with microvascular flap were performed. Intraoperative radiotherapy and postoperative radiotherapy were also delivered. Twenty-two months postoperatively, the patient is alive despite recurrence of the tumor in the neck and lung metastases. The article reviews the few series of patients and the small number of case reports of MPNSTs involving the lateral skull base that are available in the English literature describing current concept of pathophysiology, diagnosis, and management of the disease. Although MPNSTs of the lateral skull base have an adverse prognosis, an acceptable survival time and a good quality of life are possible; however, they require an early and correct diagnosis as well as an adequate and aggressive combination therapy.