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Chordoma
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Chordoma

Contributors: Brianna Caraet MD, Susan M. McDowell MD
Other Resources UpToDate PubMed

Synopsis

Causes / typical injury mechanism: Classical chordoma is a rare, slow-growing, low-to-intermediate grade primary malignant tumor of bone that is believed to be derived from remnants of notochord. 

Classic history and presentation: The most common primary sites are within the axial skeleton: the sacrum / coccyx, skull base / clivus, and mobile spine.

Prevalence: Incidence of chordomas is about 1 per 1 million, and these tumors comprise 1%-4% of all primary malignant bone tumors.
  • Age – Chordomas usually occur in patients older than 40 years (peak incidence at age 50-60 years) and rarely occur in the pediatric population.
  • Sex / gender – Chordomas are more common in men.
Pathophysiology: Pathophysiology is believed to be malignant degeneration of notochordal rests. Chordoma metastasizes mostly to the lungs and rarely to other bones.

Grade / classification system: Chordoma typically behaves in a low-to-intermediate grade manner with local recurrences being problematic after surgically treated disease.

There are several chordoma histologic variants –
  • Classical chordoma: 97% of all chordoma cases
  • Chondroid chordoma: best prognosis
  • Dedifferentiated chordoma: worst prognosis
  • Poorly differentiated chordoma: defined only recently

Codes

ICD10CM:
C41.9 – Malignant neoplasm of bone and articular cartilage, unspecified

SNOMEDCT:
50007008 – Chordoma

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Last Reviewed:03/09/2021
Last Updated:03/24/2021
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Chordoma
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