Difference between revisions of "Chondrosarcoma"
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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==== | ||
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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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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 | *S-100 positive | ||
*Keratin negative | |||
*Collagen II positive | |||
==See also== | ==See also== |
Latest revision as of 16:53, 6 February 2016
Chondrosarcoma | |
---|---|
Diagnosis in short | |
Chondrosarcoma. H&E stain. | |
| |
LM | "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 |
LM DDx | chondroblastic osteosarcoma, enchondroma (esp. for low-grade chondrosarcoma), chordoma, others |
Molecular | t(9;22) for extraskeletal myxoid chondrosarcoma |
Gross | cartilaginous appearance |
Site | hip, shoulder, soft tissue, others |
| |
Syndromes | Olier disease, Maffucci syndrome |
| |
Clinical history | adults |
Signs | mass lesion |
Prevalence | uncommon |
Prognosis | good ~75% five year survival |
Clin. DDx | enchondroma, bone tumours, soft tissue lesions |
Treatment | excision |
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 - 75% five year survival in one large data set.[1]
- 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%![2]
- Grade and stage are independent predictors of survival.[2]
Clinical/epidemiologic features:[3]
- 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).
Subtypes
Several subtypes and their relative prevalence:[2]
- 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.[2]
- Classically hip.
- Axial skeleton ~30% of cases.
- Soft tissue ~10% of cases.
Note:
- Peripheral chondrosarcoma are very rare.[4]
- Chondrosarcoma is the most common primary malignant chest wall lesion.[5]
- The classical location is anterior (costochondral arches or sternum), where it is more common than chondroma.
Microscopic
- "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.
- High grade lesions:
- Increased cellularity.
- More cellular than cartilage... but relatively paucicellular compared to other sarcomas.
- Irregular spacing of chondrocytes.
- +/-Nuclear atypia - high grade lesions.
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.[8]
DDx:
- Chordoma.
- Enchondroma.
- Synovial chondromatosis.
- Osteosarcoma - esp. chondroblastic osteosarcoma - has osteoid, may be focal.
Images
www:
Variants
Mesenchymal chondrosarcoma
Main article: Mesenchymal chondrosarcoma
Myxoid chondrosarcoma
Microscopic: Features:
DDx:
- Chondroid syringoma - These are dermal based, circumscribed and much smaller.
- Parachordoma.[10]
- Chordoma. (???)
- Myxoid liposarcoma.
- Metastatic myxoid carcinoma.
Images:
Clear cell chondrosarcoma
- Rare variant of chondrosarcoma (1.6%–5.4% of all chondrosarcomas)
- Usually a low-grade malignant tumour[11]
- 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 [12]
- 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
- Pathology outlines [3]
- Pathology outlines [4]
- Pathology outlines [5]
- Tumor library [6]
- Tumor library [7]
DDX:
- Chondroblastoma
- Giant cell tumour of bone
- Osteoblastic tumours
- Metastatic clear cell renal cell carcinoma
Outside sources: E-immunohistochemistry[8]
Extraskeletal myxoid chondrosarcoma
- Originally thought to be a variant of myxoid chondrosarcoma of bone; however, may not be a chondrosarcoma at all.[13]
- Characteristic chromosomal translocation: t(9;22) CHN-EWS.
DDx:
- Chordoma.[13]
- S-100 +ve (strong).
- EMA +ve.
- Myxoid liposarcoma.
Image:
Dedifferentiated chondrosarcoma
Clinical:
- Abysmal to poor prognosis.
Features:[16]
- Poorly differentiated (mesenchymal) malignancy.
- Well-differentiated cartilaginous component.
DDx:
- Undifferentiated pleomorphic sarcoma - no cartilaginous component.
- Other dedifferentiated tumours, e.g. dedifferentiated liposarcoma, with a minimal differentiated component.
Images:
Grading
Features:[17]
- 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 positive
- Keratin negative
- Collagen II positive
See also
References
- ↑ Damron, TA.; Ward, WG.; Stewart, A. (Jun 2007). "Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report.". Clin Orthop Relat Res 459: 40-7. doi:10.1097/BLO.0b013e318059b8c9. PMID 17414166.
- ↑ 2.0 2.1 2.2 2.3 Giuffrida, AY.; Burgueno, JE.; Koniaris, LG.; Gutierrez, JC.; Duncan, R.; Scully, SP. (May 2009). "Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database.". J Bone Joint Surg Am 91 (5): 1063-72. doi:10.2106/JBJS.H.00416. PMID 19411454.
- ↑ 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.
- ↑ Henderson, ER.; Pala, E.; Angelini, A.; Rimondi, E.; Ruggieri, P. (2013). "Dedifferentiated peripheral chondrosarcoma: a review of radiologic characteristics.". Sarcoma 2013: 505321. doi:10.1155/2013/505321. PMID 23589702.
- ↑ Somers, J.; Faber, LP. (Jul 1999). "Chondroma and chondrosarcoma.". Semin Thorac Cardiovasc Surg 11 (3): 270-7. PMID 10451259.
- ↑ IAV. 26 February 2009.
- ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 417. ISBN 978-1416002741.
- ↑ Dickson, B. 28 April 2011.
- ↑ URL: http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm. Accessed on: 29 December 2013.
- ↑ Fisher C (May 2000). "Parachordoma exists--but what is it?". Adv Anat Pathol 7 (3): 141–8. PMID 10809219.
- ↑ Corradi, D.; Bacchini, P.; Campanini, N.; Bertoni, F. (Nov 2006). "Aggressive clear cell chondrosarcomas: do distinctive characteristics exist?: a report of 4 cases.". Arch Pathol Lab Med 130 (11): 1673-9. doi:10.1043/1543-2165(2006)130[1673:ACCCDD]2.0.CO;2. PMID 17076530.
- ↑ Mokhtari, S.; Mirafsharieh, A. (2012). "Clear cell chondrosarcoma of the head and neck.". Head Neck Oncol 4: 13. doi:10.1186/1758-3284-4-13. PMID 22520362.
- ↑ 13.0 13.1 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.
- ↑ URL: http://www.cttr.org/cms/?p=736. Accessed on: 1 May 2011.
- ↑ 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.
- ↑ 16.0 16.1 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.
- ↑ 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.