The prevailing theory is that in chordomas the notochord fails to degenerate and then undergoes malignant transformation. The major failing of this theory is that normal notochord remnants have never been observed. The treatment of chordomas is difficult. Wide surgical excision is desirable but rarely feasible based on the anatomic location of the tumor. With sacrococcygeal tumors, sexual function and sphincter control may be compromised after surgery.
Radiation is used if complete resection is impossible.
Chordomas metastasize to lymph nodes, lungs, liver and bone. Chemotherapy can be used for late stage disease. Treatment and Prognosis, W.B. Saunders.