Chordoma




CLINICAL SUMMARY


Chordoma is a slow-growing malignancy with a strong predisposition for the sacrum and skull base. Because of its location, sacrococcygeal chordoma is often discovered after it has attained a large size. Resection of large tumors can be associated with a disabling loss of sacral nerve root function, while local recurrence is common due to the difficulty of obtaining wide margins. Metastasis is usually a late event.




DIAGNOSTIC FEATURES
























History


  • Unusual younger than age 40




  • Vague lower spinal pain; constipation is a late symptom



  • Symptoms often present for more than 1 year



  • Up to 30% of patients may have radiculopathy

Location


  • Almost exclusively found at the two ends of the spine



  • Anterior sacrococcygeal (50% to 60%), with S3/S4 most common; spheno-occipital less common (25% to 40%)

Margins


  • Sacrococcygeal chordoma is often missed on radiographs



  • CT demonstrates aggressive bone destruction, often large soft tissue mass

Matrix


  • Stippled calcification seen in majority of cases






IMAGING

























  • AP radiograph of a sacrococcygeal chordoma showing calcification and partial destruction of the lower sacrum ( left ).



  • Lateral radiograph shows subtle destructive process of the distal sacrum ( right ).





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