Malignant peripheral nerve sheath tumor of the scalp: case report and review of the literature.

摘要

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon malignant spindle cell tumors that account for 5% to 10% of all soft tissue sarcomas. Approximately 50% of MPNSTs arise from neurofibromas with or without neuro-fibromatosis type 1, whereas most of the remainder develop sporadically from large and medium-sized nerve trunks in deep anatomic locations on the proximal extremities or the trunk. Rarely, MPNSTs arise after radiation treatment. The tumor is considered to be derived from components of the nerve sheath such as perineural fibroblasts or Schwann cells. MPNSTs are highly aggressive with rapid invasive growth and early hematogenous dissemination. Even after radical surgical treatment MPNSTs have a dismal prognosis, with reported 5-year survival rates of less than 50%.