Difference between revisions of "Chordoma"

From Libre Pathology
Jump to navigation Jump to search
Line 73: Line 73:


===Images===
===Images===
====Case 1====
<gallery>
<gallery>
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC)
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC)
Line 78: Line 79:
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC)
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC)
Image:Chordoma - very high mag.jpg| Chordoma - very high mag. (WC)
Image:Chordoma - very high mag.jpg| Chordoma - very high mag. (WC)
</gallery>
====Case 2====
<gallery>
Image:Bone Chordoma 2 HP2.JPG|Physaliphorous cells. (SKB)
Image:Bone Chordoma 2 HP2.JPG|Physaliphorous cells. (SKB)
Image:Bone Chordoma 2 LP PA.JPG|Low power view.  Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)
Image:Bone Chordoma 2 LP PA.JPG|Low power view.  Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)
Line 85: Line 89:
Image:Bone Chordoma HP PA.JPG|Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)
Image:Bone Chordoma HP PA.JPG|Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)
</gallery>
</gallery>
www:
====www====
*[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)].
*[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)].
*[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)].

Revision as of 18:31, 14 January 2016

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.
  • 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

Case 1

  • Chordoma - low mag. (WC)

  • Chordoma - intermed. mag. (WC)

  • Chordoma - high mag. (WC)

  • Chordoma - very high mag. (WC)

Case 2

  • 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:[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.
  • Other keratins:[8]
    • 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:[9]

  • 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. ↑ 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.
  9. ↑ 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.
Retrieved from "https://librepathology.org/w/index.php?title=Chordoma&oldid=42208"