Extra-axial chordoma

Arch Pathol Lab Med. 2006 Dec;130(12):1871-4. doi: 10.5858/2006-130-1871-EC.


Chordomas are low-grade malignant tumors of bone that occur almost exclusively in the axial skeleton. Other neoplasms with a similar histologic picture but an extra-axial location have been described, including parachordoma, myxoid chondrosarcoma, and extra-axial chordoma. We herein present another case of the rare extra-axial chordoma. A 41-year-old woman developed an 8.3 cm mass in the pubic bone. The gross, microscopic, and immunohistochemical findings were identical to those of a classic chordoma. Parachordoma and myxoid chondrosarcoma were excluded from the differential diagnosis. Five previously reported cases of extra-axial chordoma were reviewed and found also to demonstrate clinical and pathologic features specific to chordoma, despite arising in an extra-axial location. Although rare, extra-axial chordoma does exist and should be recognized and managed in a similar fashion to its well-described counterpart. It must be differentiated from other histologic mimics, because the treatment and prognosis can differ significantly.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biomarkers, Tumor / analysis
  • Bone Neoplasms / chemistry
  • Bone Neoplasms / pathology*
  • Bone Neoplasms / therapy
  • Chordoma / chemistry
  • Chordoma / pathology*
  • Chordoma / therapy
  • Combined Modality Therapy
  • Disease-Free Survival
  • Female
  • Humans
  • Pubic Bone / pathology*
  • Radiotherapy, Adjuvant
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Ultrasonography


  • Biomarkers, Tumor