They are slow growing malignant neoplasms that originate from notochord remnants. These tumors display a relentless growth pattern, with more often than not multiple failed attempts at treatment. Extensive bone destruction and multiple cranial neuropathies are the hallmark of these of chordomas. Recurrences can be observed several years following treatment. Surgery is the treatment of choice.
Radiation therapy can be effective as an adjunctive treatment.
Multiple relapses are common and these are effectively managed by repeat surgery as this can significantly prolong a symptom free survival time. Chondrosarcomas Chondrosarcomas arise from cartilage remnants in the skull base as this part of the skull grows from endochondral ossification. Their malignant potential is variable and they behave in a fashion similar to chordomas. Surgical resection is the preferred method of treatment. Clinically these tumors behave in the same manner as cholesteatomas of the petrous apex.