Chordoma
- Tumor derived from persistent notochord within the sacrum and skull base, typically:
- Sacrum most commonly (~45%)
- Clivus slightly less commonly (~35%)
- Vertebral column least commonly, (~ 20%).
- The mean age of presentation is 60 y.o. and the prognosis is poor (typical survival < 10 years)
- Although the intervertebral disks contain vestigial notochord (within the nucleus pulposus), chordomas derive from the vertebral bodies: the bony portion of the spine, rather than the jelly-like disk.
- Chordomas are soft, slimy, glistening, lobulated masses with mixed fluid/gelatinous mucoid material.
- Their appearance helps us associate them with the jelly-like substance of the intervertebral disks (but remember they grow out of the vertebral bodies).
- Chordomas are infiltrative, thus they destroy the bony portion of the spine and invade surrounding neural spaces.
- Microscopically, they notably contain physaliphorous cells (vacuolated cells).
- Subtypes:
- Conventional chordoma
- Chondroid chordoma
- Dedifferentiated chordoma (rapidly fatal)
References
- “Chordoma - Conditions - Neurooncology - Specialties - UR Neurosurgery - University of Rochester Medical Center.” Accessed July 22, 2018. https://www.urmc.rochester.edu/neurosurgery/specialties/neurooncology/conditions/chordoma.aspx.
- Folpe, Andrew L., and Carrie Y. Inwards. Bone and Soft Tissue Pathology E-Book: A Volume in the Foundations in Diagnostic Pathology Series. Elsevier Health Sciences, 2009.
- IV, Griffith R. Harsh, and Francisco Vaz-Guimaraes. Chordomas and Chondrosarcomas of the Skull Base and Spine. Academic Press, 2017.
- Nikoghosyan, Anna V, Irini Karapanagiotou-Schenkel, Marc W Münter, Alexandra D Jensen, Stephanie E Combs, and Jürgen Debus. “Randomised Trial of Proton vs. Carbon Ion Radiation Therapy in Patients with Chordoma of the Skull Base, Clinical Phase III Study HIT-1-Study.” BMC Cancer 10 (November 5, 2010): 607. https://doi.org/10.1186/1471-2407-10-607.
- Shapiro, Irving M., and Makarand V. Risbud. “Transcriptional Profiling of the Nucleus Pulposus: Say Yes to Notochord.” Arthritis Research & Therapy 12 (May 20, 2010): 117. https://doi.org/10.1186/ar3003.