Pediatric Chordoma

  • Etiology:
    — From embryonic remnants of primitive notochord
    — Slow growing malignant tumor with local destruction of bone and extension into adjacent soft tissue
  • Imaging CT:
    — Location in children – clivus (60%), spine (25%), sacrum (15%)
    — Expansile often multilobulated or septated mass
    — Lytic bony destruction
    — Mass has soft tissue component as well so overall mass often much larger than area of bony destruction
    — 50% have calcifcations
  • Imaging MRI: Heterogenous
    — T1WI: Hypointense
    — T1WI post contrast: Heterogenous enhancement with honeycomb pattern sometimes
    — T2WI: Extremely hyperintense
  • DDX: Average age 10 years older than chondrosarcoma, worse prognosis than chondrosarcoma
  • Complications:
  • Treatment:
  • Clinical: Only 5% arise in children

Radiology Cases of Chordoma

Radiology Cases of Clivus Chordoma

CT and MR of clivus chordoma
Axial (above left) CT with contrast of the face shows a large expansile mass filling the sphenoid sinus that has punctate calcifications within it and that is thinning the surrounding bone. Sagittal CT (above right) shows the mass to be arising from the top of the clivus. Coronal T2 MRI (below left) shows the mass to be heterogeneously bright and to not have any intracranial extension. Axial T1 MRI with contrast (below right) show the mass does not enhance.