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

• Published in: Spine (Philadelphia, Pa. 1976) Pa. 1976), 2008-09, Vol.33 (19), p.E712-E716
• Main Authors: Moon, Sung-Jun, Lee, Jung-Kil, Seo, Bo-Ra, Kim, Jae-Hyoo, Kim, Soo-Han, Lee, Kyung-Hwa, Lee, Min-Cheol
• Format: Article
• Language: English
• Subjects: 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
• ISSN: 0362-2436; 1528-1159
• DOI: 10.1097/BRS.0b013e31817e6995

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