We report on a 42-year-old woman with malignant peripheral nerve sheath tumor (MPNST) arising from the cervical sympathetic nerve. A collar incision and partial sternotomy were performed at the second intercostal space. The mass was spindle shaped and connected to the sympathetic trunk on the cranial and caudal sides, and it compressed the left carotid sheath on the median side. After the patient's uneventful recovery from surgery, adjuvant radiotherapy was administered to the area of resection. The patient remains well 5 years after surgery with no evidence of recurrence.
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