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Chordoma

Discussion

  • rare, slow growing tumor arising from remnants of the notochord in axial skeleton of middle aged adults;
  • most occur in midline at base of skull and in the sacrococcygeal area (it is the most common primary bone tumor of the sacrum);
  • classically a slow growing lytic, anterior scaral (50%), or cervical lesion in 35%;
  • these tumors may present w/ intra-abdominal complaints & presacral mass;

Diff Dx

  • a midline mass in terminal end of sacrum may represent a chordoma;
  • lytic destructive lesion of sacrum may represent a GCT, but these usually involve younger pts and are eccentric in upper segments;
  • in females, always consider cervical cancer;

Treatment

  • complete surgical resection is required (not radiosensitive)
  • surgical excision can include upto half of sacral roots (ie all roots on one side) and still maintain bowel and bladder function;
  • recurrence rate is high, but aggressive attempts at surgical excision are indicated;
  • while cure is rare, patients typically survive 10-15 yrs following dx