Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Eur Spine J. 2012 May;21(5):897-904. doi: 10.1007/s00586-011-2093-y. Epub 2011 Dec 3.

Abstract

Background and objectives: To summarise our experience treating patients with spinal malignant peripheral nerve sheath tumours (MPNSTs).

Methods: We retrospectively reviewed the records of patients diagnosed with spinal MPNSTs who received surgical treatment from January 1998 to December 2009.

Results: Postoperative follow-up data were available for 14/16 patients with spinal MPNSTs (7 men, 7 women; median age = 44 years [range: 23-68 years]). Eight of 14 (57.1%) patients had primary and 6/14 (42.9%) recurrent MPNSTs. A total of 12/14 (85.7%) patients underwent total tumour resection, whereas 2/14 (14.3%) patients underwent subtotal tumour resection. Malignancies were graded low in 4 (28.6%) and high in 10 (71.1%) cases. A total of 12/14 (85.7%) patients experienced tumour recurrence and 10/14 (71.4%) patients died during the course of follow-up. The 0.5- 1-, 3-, and 5-year survival rates were 64.3, 48.2, 32.1, and 21.4%, respectively. Overall survival was significantly associated with tumour malignant degree (P = 0.012).

Conclusion: Diagnosis of spinal MPNSTs should be made with reference to clinical, radiological, and pathological findings. Surgical resection is the best available option for treating spinal MPNST; however, postoperative prognosis is poor.

MeSH terms

  • Adult
  • Aged
  • Female
  • Humans
  • Male
  • Middle Aged
  • Nerve Sheath Neoplasms / classification*
  • Nerve Sheath Neoplasms / mortality
  • Nerve Sheath Neoplasms / surgery*
  • Neurosurgical Procedures / methods
  • Orthopedic Procedures / methods
  • Prognosis
  • Retrospective Studies
  • Spinal Neoplasms / classification*
  • Spinal Neoplasms / mortality
  • Spinal Neoplasms / surgery*
  • Spine / diagnostic imaging
  • Spine / pathology
  • Spine / surgery
  • Survival Rate
  • Tomography, X-Ray Computed
  • Treatment Outcome