Difference between revisions of "Osteosarcoma"

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#redirect [[Chondro-osseous_tumours#Osteosarcoma]]
'''Osteosarcoma''', also known as '''osteogenic sarcoma''', a malignant [[bone]] tumour.


===General===
*Most common malignant bone tumour in children.
*May be seen in the context of [[Li-Fraumeni syndrome]].
Trivia:
*Terry Fox was afflicited by this tumour.
====Definition====
*Tumour that makes osteoid.
**Osteoid = (extracellular) organic component of bone, normally produced by osteoblasts (cells which make bone matrix).
===Gross===
Classic locations:<ref name=Ref_TN2007_OR43>{{Ref TN2007|OR43}}</ref>
*Distal femur ~ 45%.
*Proximal tibia ~ 20%.
*Proximal humerous ~ 15%.
===Microscopic===
Features:
*Cells with malignant features (e.g. nuclear membrane irregularities, marked nuclear size differences, mitoses) surrounded by delicate strands of osteoid.
**Osteoid on H&E: pink, homogenous, "glassy".
**Tumours typically very cellular - when compared to normal bone.
*Large (multinucleated) osteoclast-like giant cells may be seen.<ref>{{cite journal |author=Papalas JA, Balmer NN, Wallace C, Sangueeza OP |title=Ossifying dermatofibroma with osteoclast-like giant cells: report of a case and literature review |journal=Am J Dermatopathol |volume=31 |issue=4 |pages=379-83 |year=2009 |month=June |pmid=19461244 |doi=10.1097/DAD.0b013e3181966747 |url=}}</ref>
=====Images=====
<gallery>
Image:Osteosarcoma_-_intermed_mag.jpg | Osteosarcoma - intermed. mag. (WC)
Image:Osteosarcoma_-_very_high_mag.jpg | Osteosarcoma - very high mag. (WC)
Image:Small_cell_osteosarcoma_-_intermed_mag.jpg | Small cell osteosarcoma - intermed. mag. (WC)
Image:Small_cell_osteosarcoma_-_high_mag.jpg | Small cell osteosarcoma - high mag. (WC)
</gallery>
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2843711/figure/F3/ Lace-like osteoid of osteosarcoma (nih.gov)].<ref name=pmid20181254>{{Cite journal  | last1 = Papandreou | first1 = C. | last2 = Skopelitou | first2 = A. | last3 = Kappes | first3 = G. | last4 = Daouaher | first4 = H. | title = Primary osteosarcoma of the urinary bladder treated with external radiotherapy in a patient with a history of transitional cell carcinoma: a case report. | journal = J Med Case Rep | volume = 4 | issue =  | pages = 70 | month =  | year = 2010 | doi = 10.1186/1752-1947-4-70 | PMID = 20181254 | PMC = 2843711 }}</ref>
====Subtypes====
Subtypes:<ref name=Ref_WMSP638>{{Ref WMSP|638}}</ref><ref>URL: [http://bestpractice.bmj.com/best-practice/monograph/780/basics/classification.html http://bestpractice.bmj.com/best-practice/monograph/780/basics/classification.html]. Accessed on: 7 April 2011.</ref>
*Conventional osteosarcoma (high grade).
**Osteoblastic osteosarcoma.
**[[Fibroblastic osteosarcoma]].
**[[Chondroblastic osteosarcoma]].
*Small cell osteosarcoma.
*[[Telangiectatic osteosarcoma]].
*[[Parosteal osteosarcoma]].
*Periosteal osteosarcoma.
*[[Low-grade central osteosarcoma]].
*High-grade surface osteosarcoma.
*[[Secondary osteosarcoma]].
*Gnathic osteosarcoma - jaw bones - usually chondroblastic.
How to remember:
*Convention ''FOC'' = fibroblastic, osteogenic, chondroblastic.
*Low-grade central.
*High-grade surface.
*Parosteal.
*Periosteal.
*Small cell.
*Secondary.
*Telangiectatic.
=====Chondroblastic osteosarcoma=====
*Chondroid matrix present - may be prominent; osteoid may be a minor component.
*May be confused with [[chondrosarcoma]].
=====Fibroblastic osteosarcoma=====
*[[Undifferentiated pleomorphic sarcoma]]-like/[[MFH]]-like.
Images:
*[http://path.upmc.edu/cases/case44.html Fibroblastic osteosarcoma (upmc.edu)].
=====Low-grade central osteosarcoma=====
*Well-formed bone.
*Usu. minimal nuclear atypia.
DDx:
*[[Fibrous dysplasia]].<ref name=inwards>{{cite journal |author=Inwards, CY |title=Low-grade central osteosarcoma versus fibrous dysplasia |journal=Pathology Case Reviews |volume=6 |issue=1 |pages=22-27 |year=2001 |pmid= |doi= |url= http://journals.lww.com/pathologycasereviews/Fulltext/2001/01000/Low_Grade_Central_Osteosarcoma_Versus_Fibrous.5.aspx }}</ref>
=====Telangiectatic osteosarcoma=====
*Rare.<ref name=omim21339678>{{Cite journal  | last1 = Patibandla | first1 = MR. | last2 = Uppin | first2 = SG. | last3 = Thotakura | first3 = AK. | last4 = Panigrahi | first4 = MK. | last5 = Challa | first5 = S. | title = Primary telangiectatic osteosarcoma of occipital bone: a case report and review of literature. | journal = Neurol India | volume = 59 | issue = 1 | pages = 117-9 | month =  | year =  | doi = 10.4103/0028-3886.76891 | PMID = 21339678 }}</ref>
*Extremely vascular.
*Prognosis similar to other types of osteosarcoma.<ref name=pmid17351949>{{Cite journal  | last1 = Weiss | first1 = A. | last2 = Khoury | first2 = JD. | last3 = Hoffer | first3 = FA. | last4 = Wu | first4 = J. | last5 = Billups | first5 = CA. | last6 = Heck | first6 = RK. | last7 = Quintana | first7 = J. | last8 = Poe | first8 = D. | last9 = Rao | first9 = BN. | title = Telangiectatic osteosarcoma: the St. Jude Children's Research Hospital's experience. | journal = Cancer | volume = 109 | issue = 8 | pages = 1627-37 | month = Apr | year = 2007 | doi = 10.1002/cncr.22574 | PMID = 17351949 }}</ref>
Important radiologic DDx:
*[[Aneurysmal bone cyst]].
=====Parosteal osteosarcoma=====
*Arise from surface of bone.<ref>{{Ref WHOSTAB|279}}</ref>
*Low grade.<ref name=pmid16896870>{{Cite journal  | last1 = Carrle | first1 = D. | last2 = Bielack | first2 = SS. | title = Current strategies of chemotherapy in osteosarcoma. | journal = Int Orthop | volume = 30 | issue = 6 | pages = 445-51 | month = Dec | year = 2006 | doi = 10.1007/s00264-006-0192-x | PMID = 16896870 | PMC = 3172747 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3172747/?tool=pubmed}}</ref>
*Well-formed bone.
DDx:
*[[Fibrous dysplasia]].{{fact}}
=====Periosteal osteosarcoma=====
*Intermediate grade.<ref name=pmid16896870/>
=====Small cell osteosarcoma=====
*May mimic (other) [[small round cell tumours]].
=====Secondary osteosarcoma=====
Arises in the context of something else - causes:
*[[Paget disease of the bone]] (~80% of secondary osteosarcomas)
*Radiation (~15% of secondary osteosarcomas)).<ref>URL: [http://www.rsna.org/REG/publications/rg/afip/privateM/1997/0017/0005/1205/6.htm http://www.rsna.org/REG/publications/rg/afip/privateM/1997/0017/0005/1205/6.htm]. Accessed on: 8 April 2011.</ref>
*Prognosis often poor.<ref name=pmid16896870/>
Images:
*[http://path.upmc.edu/cases/case598.html Secondary osteosarcoma - several images (upmc.edu)].
==See also==
*[[Chondro-osseous tumours]].
*[[Bone]].
==References==
{{Reflist|2}}
[[Category:Chondro-osseous tumours]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
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