- 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
Ten Most Common Bone and Joint Tumors--Symposium: Chordomas.
Operative Treatment of Sacrococcygeal Chordoma. A Review of Twenty-one Cases.
Chordoma: a critical review of diagnosis and treatment.