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  1. It is a rare low grade slowly growing locally destructive malignant neoplasm arising from primitive notochordal remnants.
  2. It usually involved the axial skeleton, particularly extremes of spinal axis i.e. sacrococcygeal (most common) and spheno-occipital (or basi- occipital or clivus) regions and occasionally elsewhere in the spinal column (i.e. vertebrae) In adults, remenant of notochord is nucleus pulposus.
  3. Usually involves patients over 40 years and commonly present with long standing back ache and eventually causing rectal or urethral obstruction & presacral mass as tumor commonly expands anteriorly.
  4. Wide excision with radiotherapy is the treatment of choice.

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