Other tumors of bone

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

Revised: 20 October 2016, last major update June 2005

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

PubMed search: chordoma [title] bone

Cite this page: Chordoma. website. Accessed May 25th, 2018.
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
Gross description
  • Soft, gelatinous, hemorrhagic, gray tumor
Microscopic (histologic) 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
Microscopic (histologic) images
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Sacral tumor




Alcian blue

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Quiz case

Incipient chordoma


Neural type cadherin (figure 2D)

Cytology images
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Diff Quik

Positive stains
Molecular / cytogenetics description
  • Aneuploid
Electron microscopy description
Differential diagnosis