Lumbar Chordoma: A Primary Bone Tumor

Authors

Andrew N. Fleischman, BS, Department of Pathology, Anatomy, and Cell Pathology, Jefferson Medical College of Thomas Jefferson University, Philadelphia, PAFollow
Brian O'Hara, MD, Department of Pathology, Anatomy, and Cell Pathology, Jefferson Medical College of Thomas Jefferson University, Philadelphia, PAFollow

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 1^st 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

Recommended Citation