Favorable outcome after radical resection and subsequent local irradiation of malignant peripheral nerve sheath tumor in the cervical spine

A 25-year-old man presented with malignant transformation to malignant peripheral nerve sheath tumor (MPNST) in the cervical spine associated with neurofibromatosis type 1. He presented with a 3-week history of rapidly increasing weakness and numbness in all four extremities. Magnetic resonance (MR) imaging of the cervical spine demonstrated a dumbbell-shaped tumor, which compressed the spinal cord at the C2-3 level. The tumor was excised, mainly within the spinal canal to decompress the spinal cord. The histological diagnosis was benign neurofibroma. Three months after surgery, he rapidly developed progressive tetraparesis and MR imaging revealed marked regrowth of an extradural mass into the spinal canal. At reoperation, the regrown mass in the spinal canal was totally excised. The histological diagnosis revealed MPNST. He underwent radiation therapy, with a total dose of 32 Gy, for approximately 3 weeks after the second surgery, but MR imaging showed tumor regrowth within the spinal canal, and his condition deteriorated. The decision was made to remove the tumor radically, including the involved facet and extradural lesion. Posterior fusion using a pedicle screw was performed one month later. He manifested no additional neurological deficits. He has been free of relapse for 46 months. Radical resection remains the most effective treatment for MPNST, although complete removal with a clear tumor margin is often impossible in practice.