Bone
Other tumors of bone
Chordoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 20 October 2016, last major update June 2005

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed search: chordoma [title] bone

Cite this page: Chordoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bonechordoma.html. Accessed December 11th, 2016.
Definition / General
  • Rare malignant midline bone tumor arising from fetal notocord, usually within vertebral bodies, but possibly also in intervertebral discs or presacral soft tissue
  • May arise from intraosseous benign notochordal cell tumor (Am J Surg Pathol 2007;31:1573, Mod Pathol 2005;18:1005)
  • Usually males, age 40 - 60 years
  • Slow growing with repeated recurrences; late distant metastases to skin, bone, ovary (Arch Pathol Lab Med 1990;114:208)
  • Invasiveness may be due to expression of cathepsin K (Hum Pathol 2000;31:834)
  • Sites: 50% sacrococcygeal (ages 40 - 59 years), 35% spheno-occipital / clivus (particularly children, image of clivus), 15% thoracolumbar spine
  • Sacrococcygeal: sacrum destroyed by osteolytic tumor; tumor may extend into retroperitoneum, presents as palpable extrarectal mass
  • Spheno-occipital: presents as nasal, paranasal or nasopharyngeal mass involving cranial nerves
  • Posterior mediastinum: Xray presentation is well circumscribed, encapsulated soft tissue mass separate from spine (Hum Pathol 1995;26:1354)
Diagrams / Tables
Images hosted on other servers:

Clival chordoma

Radiology Images
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Sagittal MRI of sacral tumor

Prognostic Factors
  • Poor
  • Large tumor size, positive surgical margins, tumor necrosis, high proliferative activity, areas of dedifferentiation
  • Up regulation of N cadherin and down regulation of E cadherin (Am J Surg Pathol 2005;29:1422)
Case Reports
Treatment
Gross Description
  • Soft, gelatinous, hemorrhagic, gray tumor
Micro Description
  • Cords and lobules of physaliferous (having bubbles or vacuoles) cells separated by fibrous septa with extensive myxoid stroma
  • Cells may be very large, with vacuolated cytoplasm, prominent vesicular nucleus (Arch Pathol Lab Med 2004;128:1457)
  • Small tumor cells with small nucleus
  • Rare mitotic figures
Micro Images
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Sacral tumor



Stains:

Pankeratin

Synaptophysin

Alcian blue



Images hosted on other servers:

Quiz case

Incipient chordoma



Stains:

Neural type cadherin (figure 2D)

Cytology Images
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Diff Quik

Positive Stains
Molecular / Cytogenetics Description
  • Aneuploid
Electron Microscopy Description
Differential Diagnosis