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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



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.



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