Difference between revisions of "Chondrosarcoma"

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[[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]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Chondrosarcoma_(3).jpg
| Width      =
| Caption    = Chondrosarcoma. [[H&E stain]].
| Synonyms  =
| Micro      = "abnormal cartilage": +/-high grade changes - nuclear atypia (nuclear clearing, nucleoli, hyperchromasia), low/intermediate grade changes - bi-nucleation, hypochromatic enlarged nuclei, infiltration of lamellar bone ("invasion"), increased cellularity, irregular spacing of chondrocytes
| Subtypes  = chondrosarcoma not otherwise specified (NOS), juxtacortical chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma, clear cell chondrosarcoma, dedifferentiated chondrosarcoma
| LMDDx      = [[chondroblastic osteosarcoma]], [[enchondroma]] (esp. for low-grade chondrosarcoma), [[chordoma]], others
| Stains    =
| IHC        =
| EM        =
| Molecular  = t(9;22) for [[extraskeletal myxoid chondrosarcoma]]
| IF        =
| Gross      = cartilaginous appearance
| Grossing  =
| Site      = hip, shoulder, soft tissue, others
| Assdx      =
| Syndromes  = Olier disease, Maffucci syndrome
| Clinicalhx = adults
| Signs      = mass lesion
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good ~75% five year survival
| Other      =
| ClinDDx    = [[enchondroma]], [[bone tumours]], [[soft tissue lesions]]
| Tx        = excision
}}
[[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==
==General==
*Usually a good prognosis.
*Usually a good prognosis - 75% five year survival in one large data set.<ref name=pmid17414166>{{Cite journal  | last1 = Damron | first1 = TA. | last2 = Ward | first2 = WG. | last3 = Stewart | first3 = A. | title = Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. | journal = Clin Orthop Relat Res | volume = 459 | issue =  | pages = 40-7 | month = Jun | year = 2007 | doi = 10.1097/BLO.0b013e318059b8c9 | PMID = 17414166 }}</ref>
**Subtypes vary substantially - ''chondrosarcoma NOS'' and ''myxoid chondrosarcoma'' have a five year survival of ~70%, but ''mesenchymal chondrosarcoma'' only ~50%, and ''dedifferentiated chondrosarcoma'' an abysmal ~0%!<ref name=pmid19411454/> 
*Grade and stage are independent predictors of survival.<ref name=pmid19411454>{{Cite journal  | last1 = Giuffrida | first1 = AY. | last2 = Burgueno | first2 = JE. | last3 = Koniaris | first3 = LG. | last4 = Gutierrez | first4 = JC. | last5 = Duncan | first5 = R. | last6 = Scully | first6 = SP. | title = Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. | journal = J Bone Joint Surg Am | volume = 91 | issue = 5 | pages = 1063-72 | month = May | year = 2009 | doi = 10.2106/JBJS.H.00416 | PMID = 19411454 }}</ref>


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>
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>
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*May be associated with a syndrome:
*May be associated with a syndrome:
**Olier disease (multiple enchondromatosis).
**Olier disease (multiple enchondromatosis).
**Maffucci syndrome (multiple enchondromas and hemangiomas).
**Maffucci syndrome (multiple [[enchondroma]]s and [[hemangioma]]s).


Notes:
===Subtypes===
*Review article (from oncology perspective): PMID 17545802.
Several subtypes and their relative prevalence:<ref name=pmid19411454/>
*Chondrosarcoma not otherwise specified (NOS) ~83% of cases.
*Juxtacortical chondrosarcoma <1% of cases.
*Myxoid chondrosarcoma ~10% of cases.
*Mesenchymal chondrosarcoma ~4% of cases.
*Clear cell chondrosarcoma <1% of cases
*Dedifferentiated chondrosarcoma ~1% of cases.
 
==Gross==
*Appendicular skeleton ~45% of cases.<ref name=pmid19411454/>
**Classically hip.
*Axial skeleton ~30% of cases.
*Soft tissue ~10% of cases.


===Subtypes===
Note:
Several subtypes exist:
*Peripheral chondrosarcoma are very rare.<ref name=pmid23589702>{{Cite journal  | last1 = Henderson | first1 = ER. | last2 = Pala | first2 = E. | last3 = Angelini | first3 = A. | last4 = Rimondi | first4 = E. | last5 = Ruggieri | first5 = P. | title = Dedifferentiated peripheral chondrosarcoma: a review of radiologic characteristics. | journal = Sarcoma | volume = 2013 | issue =  | pages = 505321 | month =  | year = 2013 | doi = 10.1155/2013/505321 | PMID = 23589702 }}
*Chondrosarcoma not otherwise specified (NOS).
</ref>
*Juxtacortical chondrosarcoma.
*Chondrosarcoma is the most common primary malignant chest wall lesion.<ref name=pmid10451259>{{Cite journal  | last1 = Somers | first1 = J. | last2 = Faber | first2 = LP. | title = Chondroma and chondrosarcoma. | journal = Semin Thorac Cardiovasc Surg | volume = 11 | issue = 3 | pages = 270-7 | month = Jul | year = 1999 | doi =  | PMID = 10451259 }}</ref>
*Myxoid chondrosarcoma.
**The classical location is anterior (costochondral arches or sternum), where it is more common than chondroma.
*Mesenchymal chondrosarcoma.
*Clear cell chondrosarcoma.
*Dedifferentiated chondrosarcoma.


==Microscopic==
==Microscopic==
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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.
 
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====
====Myxoid chondrosarcoma====
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DDx:
DDx:
*Chondroid [[syringoma]].
*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>
*[[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]]. (???)
*[[Chordoma]]. (???)
*Myxoid liposarcoma.
*Metastatic myxoid carcinoma.
Images:
<gallery>
Image:Bone Chondrosarcoma Myxoid MP2 PA.JPG|Anastomizing chords of small neoplastic cells surround mucin pools.(SKB)
Image:Bone Chondrosarcoma Myxoid MP PA.JPG|Chords of neoplastic cells surround mucin pools. (SKB)</gallery>
*Pathology outlines [http://pathologyoutlines.com/wick/softtissue/chondrosarcomaextraskeletalmyxoidtypemicro1.jpg]
*Pathology outlines [http://pathologyoutlines.com/wick/softtissue/chondrosarcomaextraskeletalmyxoidtypemicro2.jpg]
====Clear cell chondrosarcoma====
*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 findings
*Lobules of uniform to polymorphic densely-packed large cells
*Well defined pushing borders
*Clear to intensively acidophilic granular cytoplasm cytoplasm with vacuoles
*Central nuclei with occasional prominent nucleoli
*Low mitotic rate
*Clear cell areas lack production of hyaline chondroid matrix
*Areas with osteoclast-type giant cells mixed with small trabeculae of reactive bone
*May contain conventional low-grade chondrosarcoma
*May have secondary aneurysmal bone cyst changes
<gallery>
Image:Bone Chondrosarcoma ClearCell HP PA.jpg|High grade round cells with cytoplasmic clearing. (SKB)
Image:Bone Chondrosarcoma ClearCell MP3 PA.jpg|High grade round cells with cytoplasmic clearing. (SKB)
Image:Bone Chondrosarcoma ClearCell MP4 PA.jpg|Clear cells, giant cells and bone spicules. (SKB)
Image:Bone Chondrosarcoma ClearCell MP2 PA.jpg|Somewhat hemangiopericytomatous vascular pattern with giant cells. (SKB)
Image:Bone Chondrosarcoma ClearCell MP4 PA.jpg|Clear cells, giant cells and bone spicules. (SKB)
Image:Bone Chondrosarcoma ClearCell MP PA.jpg|The lesion also had areas of more conventional chondrosarcoma. (SKB)
</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]
DDX:
*Chondroblastoma
*Giant cell tumour of bone
*Osteoblastic tumours
*Metastatic clear cell renal cell carcinoma
Outside sources:
E-immunohistochemistry[http://e-immunohistochemistry.info/web/Clear_cell_chondrosarcoma.htm]


====Extraskeletal myxoid chondrosarcoma====
====Extraskeletal myxoid chondrosarcoma====
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**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====
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DDx:
DDx:
*[[Undifferentiated pleomorphic sarcoma]] - no cartilaginous component.
*[[Undifferentiated pleomorphic sarcoma]] - no cartilaginous component.
*Other dedifferentiated tumours, e.g. [[dedifferentiated liposarcoma]],
*Other dedifferentiated tumours, e.g. [[dedifferentiated liposarcoma]], with a minimal differentiated component.


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===
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*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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