An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature

Spine (Phila Pa 1976). 2008 Sep 1;33(19):E712-6. doi: 10.1097/BRS.0b013e31817e6995.

Abstract

Study design: Case description.

Objectives: To report a rare case of intraosseous malignant peripheral nerve sheath tumors (MPNST), and review the pertinent medical literature.

Summary of background data: The spinal MPNST that develops from spinal nerve roots and secondary bony erosion is well-known entity. However, primary intraosseous MPNSTs of the spine are extremely rare.

Methods: A 41-year-old male presented with a 1-month history of radiating pain to his right shoulder and arm. Magnetic resonance images showed a large extradural mass extending from C6 to T1 with destruction of the posterior elements of C6, C7, and T1. Complete excision of the tumor and posterior stabilization were performed through a posterior approach. The tumor was noted to originate from the posterior element of C7.

Results: The histopathology was diagnostic for a MPNST. Adjuvant chemotherapy was administered after surgery. The patient remained symptom-free for 30 months after surgery without local recurrence or metastasis.

Conclusion: We report an intraosseous MPNST of the cervical spine. Complete surgical excision and adjuvant chemotherapy resulted in a good functional outcome. MPNST should be added to the differential diagnosis of primary bone tumors causing spinal cord compression.

Publication types

  • Case Reports

MeSH terms

  • 12E7 Antigen
  • Adult
  • Antigens, CD / metabolism
  • Antigens, CD34 / metabolism
  • Biomarkers, Tumor / metabolism
  • Cell Adhesion Molecules / metabolism
  • Cervical Vertebrae / diagnostic imaging
  • Cervical Vertebrae / pathology*
  • Cervical Vertebrae / surgery
  • Chemotherapy, Adjuvant
  • Combined Modality Therapy
  • Disease-Free Survival
  • Humans
  • Ki-67 Antigen / metabolism
  • Magnetic Resonance Imaging
  • Male
  • Nerve Sheath Neoplasms / complications
  • Nerve Sheath Neoplasms / metabolism
  • Nerve Sheath Neoplasms / pathology*
  • Nerve Sheath Neoplasms / therapy
  • Radiography
  • S100 Proteins / metabolism
  • Spinal Cord Compression / etiology
  • Spinal Cord Compression / pathology
  • Spinal Neoplasms / complications
  • Spinal Neoplasms / metabolism
  • Spinal Neoplasms / pathology*
  • Spinal Neoplasms / therapy
  • Vimentin / metabolism

Substances

  • 12E7 Antigen
  • Antigens, CD
  • Antigens, CD34
  • Biomarkers, Tumor
  • CD99 protein, human
  • Cell Adhesion Molecules
  • Ki-67 Antigen
  • S100 Proteins
  • Vimentin