Document Type

Poster

Publication Date

4-26-2013

Abstract

INTRODUCTION

  • Primary malignant bone tumor of embryonic notochord remnants
  • 1-4% of primary bone tumors, <0.1 per 100,000
  • Location: Sacral (50%), Skull base (35%), Vertebral column (15%)
  • Classic, chondroid (5-15%), and dedifferentiated (5%) variants
  • Most commonly in late middle age (50s to 60s)
  • Low-grade, slow growing tumor
  • But locally aggressive, high rate of local recurrence (20% in 1st year)
  • Local recurrence is most important predictor of mortality
  • Metastasis only occurs very late in disease
  • Median survival of about 6 years, less than 12 months with mets
  • 5 year survival of 70%, 10 year survival of 40%
  • Primary therapy- aggressive surgical resection (if possible)
  • New targeted therapies currently under investigation

2013 Pathology Program (2).pdf (1194 kB)
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