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, 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

Chordoma is an uncommon tumour in neuropathology.

General

  • Location: usually sacrum or clivus.
  • It is a malignant bone tumour (1-4% of all primary bone tumors).
  • Usually after age 30.

Classification

  • 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

Case 1

Case 2

Case 3

www

IHC

Features:[4][5]

  • S-100 +ve - important.
  • AE1/AE3 +ve - important.
  • Brachyury +ve - key stain.
    • Protein important for axial development, affects notochord development.[6]
    • Brachyury literally means short tail.[7]
  • EMA +ve.
  • PAX8 -ve (1 +ve (weak) of 12.[8]).
  • Other keratins:[9]
    • CK8 +ve (16/16).
    • CK19 +ve (16/16).
    • CK18 +ve/-ve (9 +ve/16).
    • CK7 -ve (1 +ve/16).
    • CK20 -ve (0 +ve/16).

Classic chordoma panel:[10]

  • EMA, AE1/AE3, CAM5.2, vimentin, S-100.

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.
  8. Sangoi, AR.; Karamchandani, J.; Lane, B.; Higgins, JP.; Rouse, RV.; Brooks, JD.; McKenney, JK. (Mar 2011). "Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases.". Mod Pathol 24 (3): 425-9. doi:10.1038/modpathol.2010.196. PMID 21102418.
  9. Naka, T.; Iwamoto, Y.; Shinohara, N.; Chuman, H.; Fukui, M.; Tsuneyoshi, M. (Jun 1997). "Cytokeratin subtyping in chordomas and the fetal notochord: an immunohistochemical analysis of aberrant expression.". Mod Pathol 10 (6): 545-51. PMID 9195570.
  10. Shen, J.; Shi, Q.; Lu, J.; Wang, DL.; Zou, TM.; Yang, HL.; Zhu, GQ. (Dec 2013). "Histological study of chordoma origin from fetal notochordal cell rests.". Spine (Phila Pa 1976) 38 (25): 2165-70. doi:10.1097/BRS.0000000000000010. PMID 24296480.