Giant malignant peripheral nerve sheath tumor of the neck in a patient with neurofibromatosis-1.

Sixteen-year-old mentally retarded male patient presented with a giant malignant peripheral nerve sheath tumor (MPNST) on his neck. Surgery was the chosen method of treatment by the help of a neurosurgeon for dissecting the mass from the nerves of the brachial plexus and a thoracic surgeon for removing the mass from the superior mediastinum. A mass of 2 kg in weight was excised gross totally. He had an early recurrence in the second month of the follow-up period despite postoperative irradiation therapy. He died of his disease due to the compression of vena cava superior.