Difference between revisions of "Chondrosarcoma"

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#redirect [[Chondro-osseous_tumours#Chondrosarcoma]]
[[Chondrosarcoma]] is a malignant tumour of [[cartilage]]. It is in the [[Chondro-osseous_tumours|Chondro-osseous grouping of tumours]] and can be lumped into the much large category of the [[soft tissue lesions]].
 
==General==
*Usually a good prognosis.
 
Clinical/epidemiologic features:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url=http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref>
*Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma).
*May be associated with a syndrome:
**Olier disease (multiple enchondromatosis).
**Maffucci syndrome (multiple enchondromas and hemangiomas).
 
Notes:
*Review article (from oncology perspective): PMID 17545802.
 
===Subtypes===
Several subtypes exist:
*Chondrosarcoma not otherwise specified (NOS).
*Juxtacortical chondrosarcoma.
*Myxoid chondrosarcoma.
*Mesenchymal chondrosarcoma.
*Clear cell chondrosarcoma.
*Dedifferentiated chondrosarcoma.
 
==Microscopic==
Features:<ref>IAV. 26 February 2009.</ref><ref name=Ref_Klatt417>{{Ref Klatt|417}}</ref>
*"Abnormal cartilage":
**+/-Nuclear atypia - high grade lesions.
***High grade lesions:
****Nuclear clearing.
****Nucleoli.
****Hyperchromasia.
***Low/intermediate grade lesions:
****Bi-nucleation.
****Hypochromatic enlarged nuclei.
****Infiltration of lamellar bone ("invasion") - not common - '''diagnostic'''.
**Increased cellularity.
***More cellular than cartilage... but relatively paucicellular compared to other sarcomas.
**Irregular spacing of chondrocytes.
 
Notes:
*Low grade chondrosarcoma are not cytologically malignant; the diagnosis rests mostly on radiologic findings.
**The exception is ''infiltration of lamellar bone'' -- this is diagnostic of chondrosarcoma.<ref>Dickson, B. 28 April 2011.</ref>
 
DDx:
*[[Chordoma]].
*[[Enchondroma]].
*[[Synovial chondromatosis]].
*[[Osteosarcoma]] - esp. [[chondroblastic osteosarcoma]] - has osteoid, may be focal.
 
====Images====
<gallery>
Image:Chondrosarcoma_(1).jpg | Chondrosarcoma - low mag. (WC)
Image:Chondrosarcoma_(2).jpg | Chondrosarcoma - high mag. (WC)
Image:Chondrosarcoma_(3).jpg | Chondrosarcoma - high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case168.html Chondrosarcoma (upmc.edu)].
*[http://www.path.utah.edu/casepath/ms%20cases/MSCase6/chondrosarcoma%20low%20grade%20sp03-9617%20g%20(Large)%20(Large).jpg Low-grade chondrosarcoma (path.utah.edu)].<ref>URL: [http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm]. Accessed on: 29 December 2013.</ref>
===Variants===
====Mesenchymal chondrosarcoma====
*Arise in soft tissue; this is where the name comes from.<ref name=pmid14161087>{{cite journal |author=Dowling EA |title=Mesenchymal chondrosarcoma |journal=J Bone Joint Surg Am |volume=46 |issue= |pages=747–54 |year=1964 |month=June |pmid=14161087 |doi= |url=http://www.ejbjs.org/cgi/reprint/46/4/747.pdf}}</ref>
*Rare variant of chondrosarcoma.
 
Microscopic:
Features:
*"White clouds in a blue sky".
 
Image:
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
 
====Myxoid chondrosarcoma====
Microscopic:
Features:
*[[Chordoma]]-like:
**[[Myxoid]] background.
**Small cells with eosinophilic cytoplasm.
 
DDx:
*Chondroid [[syringoma]].
*Parachordoma.<ref name=pmid10809219>{{cite journal |author=Fisher C |title=Parachordoma exists--but what is it? |journal=Adv Anat Pathol |volume=7 |issue=3 |pages=141–8 |year=2000 |month=May |pmid=10809219 |doi= |url=}}</ref>
*[[Chordoma]]. (???)
 
====Extraskeletal myxoid chondrosarcoma====
*Originally thought to be a variant of ''myxoid chondrosarcoma of bone''; however, may not be a chondrosarcoma at all.<ref name=pmid14657948>{{Cite journal  | last1 = Aigner | first1 = T. | last2 = Oliveira | first2 = AM. | last3 = Nascimento | first3 = AG. | title = Extraskeletal myxoid chondrosarcomas do not show a chondrocytic phenotype. | journal = Mod Pathol | volume = 17 | issue = 2 | pages = 214-21 | month = Feb | year = 2004 | doi = 10.1038/modpathol.3800036 | PMID = 14657948 | URL = http://www.nature.com/modpathol/journal/v17/n2/full/3800036a.html  }}</ref>
*Characteristic [[chromosomal translocation]]: t(9;22) CHN-EWS.
 
DDx:
*Chordoma.<ref name=pmid14657948/>
**S-100 +ve (strong).
**EMA +ve.
 
Image:
*[http://www.cttr.org/large/03113.jpg Extraskeletal myxoid chondrosarcoma (cttr.org)].<ref>URL: [http://www.cttr.org/cms/?p=736 http://www.cttr.org/cms/?p=736]. Accessed on: 1 May 2011.</ref>
 
====Dedifferentiated chondrosarcoma====
Clinical:
*Abysmal to poor prognosis.
**In one series (22 patients) 5-year survival ~20%.<ref>{{Cite journal  | last1 = Mitchell | first1 = AD. | last2 = Ayoub | first2 = K. | last3 = Mangham | first3 = DC. | last4 = Grimer | first4 = RJ. | last5 = Carter | first5 = SR. | last6 = Tillman | first6 = RM. | title = Experience in the treatment of dedifferentiated chondrosarcoma. | journal = J Bone Joint Surg Br | volume = 82 | issue = 1 | pages = 55-61 | month = Jan | year = 2000 | doi =  | PMID = 10697315 | URL = http://www.jbjs.org.uk/cgi/pmidlookup?view=long&pmid=10697315 }}</ref>
**All dead in two years in another series (25 patients).<ref name=pmid17653766/>
 
Features:<ref name=pmid17653766>{{Cite journal  | last1 = Sopta | first1 = J. | last2 = Dordević | first2 = A. | last3 = Tulić | first3 = G. | last4 = Mijucić | first4 = V. | title = Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases. | journal = J Cancer Res Clin Oncol | volume = 134 | issue = 2 | pages = 147-52 | month = Feb | year = 2008 | doi = 10.1007/s00432-007-0262-5 | PMID = 17653766 }}</ref>
#Poorly differentiated (mesenchymal) malignancy.
#Well-differentiated cartilaginous component.
 
Images:
*[http://path.upmc.edu/cases/case118/micro.html Dedifferentiated chondrosarcoma (upmc.edu)].
 
===Grading===
Features:<ref name=Ref_WMSP643>{{Ref WMSP|643}}</ref>
*Grade I: mild-to-moderate increase of cellularity +/- binucleated cells.
*Grade II: between Grade I and Grade III.
*Grade III: nuclear pleomorphism, mitoses common.
 
==IHC==
*S-100 -ve. (???)
 
==See also==
*[[Chondro-osseous tumours]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Chondro-osseous tumours]]

Revision as of 02:33, 30 December 2013

Chondrosarcoma is a malignant tumour of cartilage. It is in the Chondro-osseous grouping of tumours and can be lumped into the much large category of the soft tissue lesions.

General

  • Usually a good prognosis.

Clinical/epidemiologic features:[1]

  • Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma).
  • May be associated with a syndrome:
    • Olier disease (multiple enchondromatosis).
    • Maffucci syndrome (multiple enchondromas and hemangiomas).

Notes:

  • Review article (from oncology perspective): PMID 17545802.

Subtypes

Several subtypes exist:

  • Chondrosarcoma not otherwise specified (NOS).
  • Juxtacortical chondrosarcoma.
  • Myxoid chondrosarcoma.
  • Mesenchymal chondrosarcoma.
  • Clear cell chondrosarcoma.
  • Dedifferentiated chondrosarcoma.

Microscopic

Features:[2][3]

  • "Abnormal cartilage":
    • +/-Nuclear atypia - high grade lesions.
      • High grade lesions:
        • Nuclear clearing.
        • Nucleoli.
        • Hyperchromasia.
      • Low/intermediate grade lesions:
        • Bi-nucleation.
        • Hypochromatic enlarged nuclei.
        • Infiltration of lamellar bone ("invasion") - not common - diagnostic.
    • Increased cellularity.
      • More cellular than cartilage... but relatively paucicellular compared to other sarcomas.
    • Irregular spacing of chondrocytes.

Notes:

  • Low grade chondrosarcoma are not cytologically malignant; the diagnosis rests mostly on radiologic findings.
    • The exception is infiltration of lamellar bone -- this is diagnostic of chondrosarcoma.[4]

DDx:

Images

  • Chondrosarcoma - low mag. (WC)

  • Chondrosarcoma - high mag. (WC)

  • Chondrosarcoma - high mag. (WC)

www:

Variants

Mesenchymal chondrosarcoma

  • Arise in soft tissue; this is where the name comes from.[6]
  • Rare variant of chondrosarcoma.

Microscopic: Features:

  • "White clouds in a blue sky".

Image:

Myxoid chondrosarcoma

Microscopic: Features:

  • Chordoma-like:
    • Myxoid background.
    • Small cells with eosinophilic cytoplasm.

DDx:

Extraskeletal myxoid chondrosarcoma

  • Originally thought to be a variant of myxoid chondrosarcoma of bone; however, may not be a chondrosarcoma at all.[8]
  • Characteristic chromosomal translocation: t(9;22) CHN-EWS.

DDx:

  • Chordoma.[8]
    • S-100 +ve (strong).
    • EMA +ve.

Image:

Dedifferentiated chondrosarcoma

Clinical:

  • Abysmal to poor prognosis.
    • In one series (22 patients) 5-year survival ~20%.[10]
    • All dead in two years in another series (25 patients).[11]

Features:[11]

  1. Poorly differentiated (mesenchymal) malignancy.
  2. Well-differentiated cartilaginous component.

Images:

Grading

Features:[12]

  • Grade I: mild-to-moderate increase of cellularity +/- binucleated cells.
  • Grade II: between Grade I and Grade III.
  • Grade III: nuclear pleomorphism, mitoses common.

IHC

  • S-100 -ve. (???)

See also

References

  1. ↑ Skubitz KM, D'Adamo DR (November 2007). "Sarcoma". Mayo Clin. Proc. 82 (11): 1409–32. PMID 17976362. http://www.mayoclinicproceedings.com/content/82/11/1409.long.
  2. ↑ IAV. 26 February 2009.
  3. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 417. ISBN 978-1416002741.
  4. ↑ Dickson, B. 28 April 2011.
  5. ↑ URL: http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm. Accessed on: 29 December 2013.
  6. ↑ Dowling EA (June 1964). "Mesenchymal chondrosarcoma". J Bone Joint Surg Am 46: 747–54. PMID 14161087. http://www.ejbjs.org/cgi/reprint/46/4/747.pdf.
  7. ↑ Fisher C (May 2000). "Parachordoma exists--but what is it?". Adv Anat Pathol 7 (3): 141–8. PMID 10809219.
  8. ↑ 8.0 8.1 Aigner, T.; Oliveira, AM.; Nascimento, AG. (Feb 2004). "Extraskeletal myxoid chondrosarcomas do not show a chondrocytic phenotype.". Mod Pathol 17 (2): 214-21. doi:10.1038/modpathol.3800036. PMID 14657948.
  9. ↑ URL: http://www.cttr.org/cms/?p=736. Accessed on: 1 May 2011.
  10. ↑ Mitchell, AD.; Ayoub, K.; Mangham, DC.; Grimer, RJ.; Carter, SR.; Tillman, RM. (Jan 2000). "Experience in the treatment of dedifferentiated chondrosarcoma.". J Bone Joint Surg Br 82 (1): 55-61. PMID 10697315.
  11. ↑ 11.0 11.1 Sopta, J.; Dordević, A.; Tulić, G.; Mijucić, V. (Feb 2008). "Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases.". J Cancer Res Clin Oncol 134 (2): 147-52. doi:10.1007/s00432-007-0262-5. PMID 17653766.
  12. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 643. ISBN 978-0781765275.
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