Difference between revisions of "Chondrosarcoma"

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Chondrosarcoma (view source)

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| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Chondrosarcoma_(3).jpg
| Image      = Chondrosarcoma_(3).jpg
| Width      =
| Width      =  
| Caption    = Chondrosarcoma. [[H&E stain]].
| Caption    = Chondrosarcoma. [[H&E stain]].
| Synonyms  =
| Synonyms  =
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Image:Chondrosarcoma_(2).jpg | Chondrosarcoma - high mag. (WC)
Image:Chondrosarcoma_(2).jpg | Chondrosarcoma - high mag. (WC)
Image:Chondrosarcoma_(3).jpg | Chondrosarcoma - high mag. (WC)
Image:Chondrosarcoma_(3).jpg | Chondrosarcoma - high mag. (WC)
Image:Bone Chondrosarcoma Grade2 MP PA.jpg|Grade 2 chondrosarcoma, low power showing bone permeation (SKB)
Image:Bone Chondrosarcoma Grade2 HP PA copy.jpg|Chondrocytic hyperchromasia and atypicality in this photo of a Grade 2 chondrosarcoma. (SKB)
Image:Bone Chondrosarcoma Grade2 MP3 PA.JPG|Lobule of neoplastic Grade 2 cartilage with some bone permeation and calcification at the top of the photo.(SKB)
Image:Bone Chondrosarcoma Grade2 HP.JPG|Lobule of neoplastic Grade 2 cartilage with atypia and bone permeation. (SKB)
Image:Bone Chondrosarcoma Grade3 MP PA.JPG|Grade 3 - very cellular neoplastic cartilage with high grade nuclear atypia. (SKB)
</gallery>
</gallery>
www:
www:
*[http://path.upmc.edu/cases/case168.html Chondrosarcoma (upmc.edu)].
*[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>
*[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===
===Variants===
====[[Mesenchymal chondrosarcoma]]====
====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>
{{Main|Mesenchymal chondrosarcoma}}
*Rare variant of chondrosarcoma.


====Myxoid chondrosarcoma====
Microscopic:
Microscopic:
Features:
Features:
*"White clouds in a blue sky".
*[[Chordoma]]-like:
**[[Myxoid]] background.
**Small cells with eosinophilic cytoplasm.


Image:
DDx:
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
*Chondroid [[syringoma]] - These are dermal based, circumscribed and much smaller.
*[[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]]. (???)
*Myxoid liposarcoma.
*Metastatic myxoid carcinoma.


==General==
Images:
<gallery>
*Rare variant of chondrosarcoma.
Image:Bone Chondrosarcoma Myxoid MP2 PA.JPG|Anastomizing chords of small neoplastic cells surround mucin pools.(SKB)
*2–10% of primary chondrosarcomas
Image:Bone Chondrosarcoma Myxoid MP PA.JPG|Chords of neoplastic cells surround mucin pools. (SKB)</gallery>
*Adolescents and young adults
*Pathology outlines [http://pathologyoutlines.com/wick/softtissue/chondrosarcomaextraskeletalmyxoidtypemicro1.jpg]
*Female predilection
*Pathology outlines [http://pathologyoutlines.com/wick/softtissue/chondrosarcomaextraskeletalmyxoidtypemicro2.jpg]
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meminges)
*Conceptualized as originating from a pleuripotential mesenchymal cell with foci recapitulating enchondral ossification.
*The small cells appear to be an undifferentiated cartilage stem cell which “differentiate” into benign cartilage <ref>{{Cite journal  | last1 = Fanburg-Smith | first1 = JC. | last2 = Auerbach | first2 = A. | last3 = Marwaha | first3 = JS. | last4 = Wang | first4 = Z. | last5 = Rushing | first5 = EJ. | title = Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 653-62 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.11.006 | PMID = 20138330 }}
</ref>.
*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>


==Gross==  
====Clear cell chondrosarcoma====
Pink and fleshy with foci of calcification.
*Rare variant of chondrosarcoma (1.6%–5.4% of all chondrosarcomas)
*Usually a low-grade malignant tumour<ref>{{Cite journal  | last1 = Corradi | first1 = D. | last2 = Bacchini | first2 = P. | last3 = Campanini | first3 = N. | last4 = Bertoni | first4 = F. | title = Aggressive clear cell chondrosarcomas: do distinctive characteristics exist?: a report of 4 cases. | journal = Arch Pathol Lab Med | volume = 130 | issue = 11 | pages = 1673-9 | month = Nov | year = 2006 | doi = 10.1043/1543-2165(2006)130[1673:ACCCDD]2.0.CO;2 | PMID = 17076530 }}</ref>
*Younger age than conventional chondrosarcoma
*Teens to 40s; more common in males
*Epiphyses of long tubular bones; proximal femur or humerus
*Rarely head and neck <ref>{{Cite journal  | last1 = Mokhtari | first1 = S. | last2 = Mirafsharieh | first2 = A. | title = Clear cell chondrosarcoma of the head and neck. | journal = Head Neck Oncol | volume = 4 | issue =  | pages = 13 | month =  | year = 2012 | doi = 10.1186/1758-3284-4-13 | PMID = 22520362 }}</ref>
*Malignant counterpart of chondroblastoma?


==Microscopic==
Microscopic findings
*"White clouds in a blue sky".
*Lobules of uniform to polymorphic densely-packed large cells
*Malignant tumor with a characteristic biphasic pattern
*Well defined pushing borders
**Cellular poorly differentiated small round blue cells
*Clear to intensively acidophilic granular cytoplasm cytoplasm with vacuoles
**Islands of well-differentiated hyaline cartilage
*Central nuclei with occasional prominent nucleoli
***Progressive maturation of cartilage towards the center
*Low mitotic rate
***Central calcification or bone formation
*Clear cell areas lack production of hyaline chondroid matrix
*Areas with osteoclast-type giant cells mixed with small trabeculae of reactive bone
==IHC==
*May contain conventional low-grade chondrosarcoma
*SOX9 (positive in small cells and chondrocytes) <ref>{{Cite journal  | last1 = Pang | first1 = ZG. | last2 = He | first2 = XZ. | last3 = Wu | first3 = LY. | last4 = Wei | first4 = W. | last5 = Liu | first5 = XY. | last6 = Liao | first6 = DY. | last7 = Li | first7 = FY. | last8 = Zhang | first8 = XL. | title = [Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 40 | issue = 6 | pages = 368-72 | month = Jun | year = 2011 | doi =  | PMID = 21914343 }}</ref>
*May have secondary aneurysmal bone cyst changes
*S100 (positive in chondrocytes not in small cells)
*Osteocalcin (negative in small cells)


==Molecular==
t(8;8)(q21.1;q13.3) HEY1-NCOA2 <ref>{{Cite journal  | last1 = Panagopoulos | first1 = I. | last2 = Gorunova | first2 = L. | last3 = Bjerkehagen | first3 = B. | last4 = Boye | first4 = K. | last5 = Heim | first5 = S. | title = Chromosome aberrations and HEY1-NCOA2 fusion gene in a mesenchymal chondrosarcoma. | journal = Oncol Rep | volume = 32 | issue = 1 | pages = 40-4 | month = Jul | year = 2014 | doi = 10.3892/or.2014.3180 | PMID = 24839999 }}</ref>
==Photos==
<gallery>
<gallery>
*Image:Bone Chondrosarcoma Mesenchymal MP5 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell HP PA.jpg|High grade round cells with cytoplasmic clearing. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal MP PA.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell MP3 PA.jpg|High grade round cells with cytoplasmic clearing. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal LP2 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell MP4 PA.jpg|Clear cells, giant cells and bone spicules. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal HP5 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell MP2 PA.jpg|Somewhat hemangiopericytomatous vascular pattern with giant cells. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal MP2 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell MP4 PA.jpg|Clear cells, giant cells and bone spicules. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal MP7.JPG|Islands of cartilage in a background of small blue cells.(SKB)
Image:Bone Chondrosarcoma ClearCell MP PA.jpg|The lesion also had areas of more conventional chondrosarcoma. (SKB)
*Image:Bone Chondrosarcoma Mesenchymal HP3 PA.JPG|Small blue cells predominate.(SKB)
</gallery>
</gallery>
*Pathology outlines [http://pathologyoutlines.com/wick/chondrosarcoma%20clear%20cell%20type%20micro7.jpg]
*Pathology outlines [http://pathologyoutlines.com/wick/chondrosarcoma%20clear%20cell%20type%20micro8.jpg]
*Pathology outlines [http://pathologyoutlines.com/wick/chondrosarcoma%20clear%20cell%20type%20micro2.jpg]
*Tumor library [http://www.tumorlibrary.com/case/images/1521.jpg]
*Tumor library [http://www.tumorlibrary.com/case/images/1522.jpg]


==See also==
DDX:
Image:
*Chondroblastoma
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
*Giant cell tumour of bone
*Osteoblastic tumours
==References==
*Metastatic clear cell renal cell carcinoma
{{Reflist|1}}
 
====Myxoid chondrosarcoma====
Microscopic:
Features:
*[[Chordoma]]-like:
**[[Myxoid]] background.
**Small cells with eosinophilic cytoplasm.


DDx:
Outside sources:
*Chondroid [[syringoma]].
E-immunohistochemistry[http://e-immunohistochemistry.info/web/Clear_cell_chondrosarcoma.htm]
*[[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====
====Extraskeletal myxoid chondrosarcoma====
Line 180: Line 181:
**S-100 +ve (strong).
**S-100 +ve (strong).
**EMA +ve.
**EMA +ve.
*Myxoid liposarcoma.


Image:
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>
*[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>
<gallery>
Image:SoftTissue ExtraskeletalMyxoidChondrosarcoma MP CTR.jpg|Strands of atypical cells suspended in mucin. (SKB)
Image:SoftTissue ExtraskeletalMyxoidChondrosarcoma HP CTR.jpg|Strands of atypical cells suspended in mucin. (SKB)
</gallery>


====Dedifferentiated chondrosarcoma====
====Dedifferentiated chondrosarcoma====
Line 200: Line 206:
Images:
Images:
*[http://path.upmc.edu/cases/case118/micro.html Dedifferentiated chondrosarcoma (upmc.edu)].
*[http://path.upmc.edu/cases/case118/micro.html Dedifferentiated chondrosarcoma (upmc.edu)].
<gallery>
Image:Bone Chondrosarcoma Dedifferentiated PA copy.jpg|A lobule of cartilagenous chondrosarcoma on the left; high grade sarcoma on the right. (SKB)
Image:Bone Chondrosarcoma Dedifferentiated HP PA.jpg|High grade sarcoma on the left and upper; malignant cartilage right and lower. (SKB)
</gallery>


===Grading===
===Grading===
Line 206: Line 216:
*Grade II: between Grade I and Grade III.
*Grade II: between Grade I and Grade III.
*Grade III: nuclear pleomorphism, mitoses common.
*Grade III: nuclear pleomorphism, mitoses common.
<gallery>
Image:Bone Chondrosarcoma Grade1 HP2 PA.JPG|Grade 1 - Somewhat cellular cartilage with binucleation.(SKB)
Image:Bone Chondrosarcoma Grade2 HP PA.jpg|Grade 2 - Very cellular cartilage with obvious hyperchromasia and nuclear atypia. (SKB)
Image:Bone Chondrosarcoma Grade3 HP PA.JPG|Grade 3 - Even more cellular neoplastic cartilage with high grade nuclear atypia. (SKB) </gallery>


==IHC==
==IHC==
*S-100 -ve. (???)
*S-100 positive
*Keratin negative
*Collagen II positive


==See also==
==See also==
Michael
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