Favorable Outcome After Radical Resection and Subsequent Local Irradiation of Malignant Peripheral Nerve Sheath Tumor in the Cervical Spine

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  • SASAMORI Toru
    Department of Neurosurgery, Hokkaido University Graduate School of Medicine
  • HIDA Kazutoshi
    Department of Neurosurgery, Hokkaido University Graduate School of Medicine
  • YANO Shunsuke
    Department of Neurosurgery, Sapporo Azabu Neurosurgical Hospital
  • AOYAMA Takeshi
    Department of Neurosurgery, Hokkaido University Graduate School of Medicine
  • ASANO Takeshi
    Department of Neurosurgery, Hokkaido University Graduate School of Medicine
  • KUBOTA Kanako
    Department of Pathology, Hokkaido University Hospital
  • ITO Manabu
    Department of Advanced Medicine for Spine and Spinal Cord Disorders, Hokkaido University Graduate School of Medicine
  • ABUMI Kuniyoshi
    Department of Spinal Reconstruction, Hokkaido University Graduate School of Medicine
  • IWASAKI Yoshinobu
    Department of Neurosurgery, Sapporo Azabu Neurosurgical Hospital
  • SAITO Hisatoshi
    Department of Neurosurgery, Sapporo Azabu Neurosurgical Hospital
  • HOUKIN Kiyohiro
    Department of Neurosurgery, Hokkaido University Graduate School of Medicine

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  • —Case Report—

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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.<br>

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