Chordoma is a rare slow-growing malignant neoplasm thought to arise from cellular remnants of the notochord. There are three histological variants of chordoma: classical, chondroid and dedifferentiated.

 Chordomas can arise anywhere along the neuraxis. The two most common locations are cranially at the clivus and at the sacrococyccygeal region of the spine.

 In the United States, the annual incidence of chordoma is approximately 1 in one million. The average 10-year tumor free survival rate is less than 50%. Aggressive surgical resection followed by radiation therapy offers the best chance of long-term control. There are no drugs currently approved to treat chordoma, however chordoma has been shown to have a limited response to the PDGFR inhibitor Imatinib.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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