Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum.

Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.