Chordoma

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Chordoma
Diagnosis in short

Chordoma. HPS stain.
LM physaliphorous cells (also bubble cells) - very large clear bubble with a sharp border, bubble does not compress nucleus; islands of cells surrounded by fibrous tissue; myxoid background
LM DDx chondrosarcoma, myxoid lesions, parachordoma
IHC S-100 +ve, AE1/AE3 +ve, Brachyury +ve, EMA +ve
Gross myxoid
Site sacrum or clivus
Prevalence uncommon

Chordoma is an uncommon tumour in neuropathology.

General

  • Location: usually sacrum or clivus.
  • It is a bone tumour.

Gross

  • Soft, gelatinous, lobulated.[1]

DDx:

  • Bony metastasis (mucinous carcinoma) - typically multifocal.

Image:

Microscopic

Features:[2]

  • Architecture: islands of cells surrounded by fibrous tissue.
    • Also described as "lobulated" architecture; may not be apparent.
  • Myxoid background - grey extracellular material, variable amount present.
  • Mixed cell population:
    1. Abundant eosinophilic cytoplasm.
    2. Physaliphorous cells or bubble cells - key feature.
      • Have a very large clear bubble with a sharp border; bubble does not compress nucleus - nucleus may be in bubble.

DDx:

  • Chondrosarcoma - negative for EMA and cytokeratins. Beware of 'chondroid' chordoma.
  • Myxoid lesions.
    • Myxopapillary ependymoma.
    • Myxoid liposarcoma - negative for EMA and cytokeratins.
  • 'Choroid lesions'
    • Choroid meningioma.
    • Choroid glioma - location, location, location.
    • Large notochordal rest - only evidence of destructive growth can identify a chordoma.
  • Metastasis
    • Metastatic signet ring cell adenocarcinoma - negative for S100 and brachyury (history!)
    • Metastatic clear cell renal cell carcinoma - negative for S100 and brachyury (history!)
  • Parachordoma - extremely rare.

Images

  • Chordoma - low mag. (WC)

  • Chordoma - intermed. mag. (WC)

  • Chordoma - high mag. (WC)

  • Chordoma - very high mag. (WC)

  • Physaliphorous cells. (SKB)

  • Low power view. Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)

  • Physaliphorous cells. (SKB)

  • Physaliphorous cells. (SKB)

  • Physaliphorous cells.(SKB)

  • Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)

www:

IHC

Features:[3][4]

  • S-100 +ve.
  • AE1/AE3 +ve.
  • Brachyury +ve -- key stain.
    • Protein important for axial development, affects notochord development.[5]
    • Brachyury literally means short tail.[6]
  • EMA +ve.

Key points:

  • Brachyury is not a commonly stocked antibody.
  • Chordoma will be S100 AND Epithelial marker positive.
  • Many other items in the DDX will be either S100 OR Epithelial marker positive.

See also

References

  1. ↑ URL: http://www.histopathology-india.net/Chordoma.htm. Accessed on: 12 April 2012.
  2. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 184. ISBN 978-0470519035.
  3. ↑ URL: http://path.upmc.edu/cases/case312/micro.html. Accessed on: 14 January 2012.
  4. ↑ Coindre, JM.; Rivel, J.; Trojani, M.; De Mascarel, I.; De Mascarel, A. (Sep 1986). "Immunohistological study in chordomas.". J Pathol 150 (1): 61-3. doi:10.1002/path.1711500110. PMID 2431128.
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601397
  6. ↑ URL: http://www.jstor.org/pss/86845. Accessed on: 18 May 2010.
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