Chordoma

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Chordoma is an uncommon tumour in neuropathology.

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, chordoid lesions (e.g. chordoid glioma, chordoid meningioma), metastasis (e.g. clear cell renal cell carcinoma)
IHC S-100 +ve, AE1/AE3 +ve, Brachyury +ve, EMA +ve
Gross myxoid
Site sacrum or clivus

Prevalence uncommon

General

  • Location: usually sacrum or clivus.
  • It is a malignant bone tumour (1-4% of all primary bone tumors).
  • Usually after age 30.
  • Comes in three flavours:
    • Chordoma, NOS (ICD-O: 9370/3)
    • Chondroid chordoma (ICD-O: 9371/3)
    • Dedifferentiated chordoma (ICD-O: 9372/3)

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:

Images

www:

IHC

Features:[4][5]

  • S-100 +ve.
  • AE1/AE3 +ve.
  • Brachyury +ve -- key stain.
    • Protein important for axial development, affects notochord development.[6]
    • Brachyury literally means short tail.[7]
  • 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. Zarghouni, M.; Vandergriff, C.; Layton, KF.; McGowan, JB.; Coimbra, C.; Bhakti, A.; Opatowsky, MJ. (Jul 2012). "Chordoid glioma of the third ventricle.". Proc (Bayl Univ Med Cent) 25 (3): 285-6. PMID 22754136.
  4. URL: http://path.upmc.edu/cases/case312/micro.html. Accessed on: 14 January 2012.
  5. 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.
  6. Online 'Mendelian Inheritance in Man' (OMIM) 601397
  7. URL: http://www.jstor.org/pss/86845. Accessed on: 18 May 2010.