Difference between revisions of "Osteosarcoma"

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'''Osteosarcoma''', also known as '''osteogenic sarcoma''', a malignant [[bone]] tumour.
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Osteosarcoma_-_very_high_mag.jpg
| Width      =
| Caption    = Osteosarcoma. [[H&E stain]].
| Micro      = cells with malignant features (e.g. nuclear membrane irregularities, marked nuclear size differences, mitoses) surrounded by delicate strands of osteoid
| Subtypes  = conventional osteosarcoma (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
| LMDDx      = [[chondrosarcoma]], [[phosphaturic mesenchymal tumour, mixed connective tissue type]], [[undifferentiated pleomorphic sarcoma]] (for fibroblastic osteosarcoma), [[aneurysmal bone cyst]] (for telangiectatic osteosarcoma), [[fibrous dysplasia]], [[small round cell tumours]] (for small cell osteosarcoma)
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[bone]]
| Assdx      =
| Syndromes  = [[Li-Fraumeni syndrome]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    = [[aneurysmal bone cyst]],[[fibrous dysplasia]], [[Ewing sarcoma]]
}}
'''Osteosarcoma''', also known as '''osteogenic sarcoma''', is a malignant [[bone]] tumour. It is grouped with the [[chondro-osseous tumours]].


===General===
==General==
*Most common malignant bone tumour in children.
*Most common malignant bone tumour in children.
*May be seen in the context of [[Li-Fraumeni syndrome]].
*May be seen in the context of [[Li-Fraumeni syndrome]].
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*Terry Fox was afflicited by this tumour.
*Terry Fox was afflicited by this tumour.


====Definition====
===Definition===
*Tumour that makes osteoid.
*Tumour that makes osteoid.
**Osteoid = (extracellular) organic component of bone, normally produced by osteoblasts (cells which make bone matrix).
**Osteoid = (extracellular) organic component of bone, normally produced by osteoblasts (cells which make bone matrix).


===Gross===
==Gross==
Classic locations:<ref name=Ref_TN2007_OR43>{{Ref TN2007|OR43}}</ref>
Classic locations:<ref name=Ref_TN2007_OR43>{{Ref TN2007|OR43}}</ref>
*Distal femur ~ 45%.  
*Distal femur ~ 45%.  
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*Proximal humerous ~ 15%.
*Proximal humerous ~ 15%.


===Microscopic===
==Microscopic==
Features:
Features:
*Cells with malignant features (e.g. nuclear membrane irregularities, marked nuclear size differences, mitoses) surrounded by delicate strands of osteoid.
*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".
**Osteoid on H&E: pink, homogenous, "glassy".
**Tumours typically very cellular - when compared to normal bone.
**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>
*+/-Large (multinucleated) osteoclast-like giant cells.<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=====
DDx:
*[[Chondrosarcoma]].
*[[Phosphaturic mesenchymal tumour, mixed connective tissue type]].
*Others.
 
====Images====
<gallery>
<gallery>
Image:Osteosarcoma - low mag.jpg|Osteosarcoma - low mag. (WC)
Image:Osteosarcoma_-_intermed_mag.jpg | Osteosarcoma - intermed. mag. (WC)
Image:Osteosarcoma_-_intermed_mag.jpg | Osteosarcoma - intermed. mag. (WC)
Image: Osteosarcoma - high mag.jpg|Osteosarcoma - high mag. (WC)
Image:Osteosarcoma_-_very_high_mag.jpg | Osteosarcoma - very high 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_-_intermed_mag.jpg | Small cell osteosarcoma - intermed. mag. (WC)
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*[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>
*[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===
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>
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).
*Conventional osteosarcoma (high grade).
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*Telangiectatic.
*Telangiectatic.


=====Chondroblastic osteosarcoma=====
====Chondroblastic osteosarcoma====
*Chondroid matrix present - may be prominent; osteoid may be a minor component.
*Chondroid matrix present - may be prominent; osteoid may be a minor component.
*May be confused with [[chondrosarcoma]].
*May be confused with [[chondrosarcoma]].


=====Fibroblastic osteosarcoma=====
====Fibroblastic osteosarcoma====
*[[Undifferentiated pleomorphic sarcoma]]-like/[[MFH]]-like.
*[[Undifferentiated pleomorphic sarcoma]]-like/[[MFH]]-like.


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*[http://path.upmc.edu/cases/case44.html Fibroblastic osteosarcoma (upmc.edu)].
*[http://path.upmc.edu/cases/case44.html Fibroblastic osteosarcoma (upmc.edu)].


=====Low-grade central osteosarcoma=====
====Low-grade central osteosarcoma====
*Well-formed bone.
*Well-formed bone.
*Usu. minimal nuclear atypia.
*Usu. minimal nuclear atypia.
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*[[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>
*[[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=====
====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>
*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.
*Extremely vascular.
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*[[Aneurysmal bone cyst]].
*[[Aneurysmal bone cyst]].


=====Parosteal osteosarcoma=====
====Parosteal osteosarcoma====
*Arise from surface of bone.<ref>{{Ref WHOSTAB|279}}</ref>
*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>
*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>
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*[[Fibrous dysplasia]].{{fact}}
*[[Fibrous dysplasia]].{{fact}}


=====Periosteal osteosarcoma=====
====Periosteal osteosarcoma====
*Intermediate grade.<ref name=pmid16896870/>
*Intermediate grade.<ref name=pmid16896870/>


=====Small cell osteosarcoma=====
====Small cell osteosarcoma====
*May mimic (other) [[small round cell tumours]].
*May mimic (other) [[small round cell tumours]].


=====Secondary osteosarcoma=====
====Secondary osteosarcoma====
Arises in the context of something else - causes:
Arises in the context of something else - causes:
*[[Paget disease of the bone]] (~80% of secondary osteosarcomas)
*[[Paget disease of the bone]] (~80% of secondary osteosarcomas)

Latest revision as of 19:59, 12 June 2014

Osteosarcoma
Diagnosis in short

Osteosarcoma. H&E stain.
LM cells with malignant features (e.g. nuclear membrane irregularities, marked nuclear size differences, mitoses) surrounded by delicate strands of osteoid
Subtypes conventional osteosarcoma (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
LM DDx chondrosarcoma, phosphaturic mesenchymal tumour, mixed connective tissue type, undifferentiated pleomorphic sarcoma (for fibroblastic osteosarcoma), aneurysmal bone cyst (for telangiectatic osteosarcoma), fibrous dysplasia, small round cell tumours (for small cell osteosarcoma)
Site bone
Syndromes Li-Fraumeni syndrome
Prevalence uncommon
Clin. DDx aneurysmal bone cyst,fibrous dysplasia, Ewing sarcoma

Osteosarcoma, also known as osteogenic sarcoma, is a malignant bone tumour. It is grouped with the chondro-osseous tumours.

General

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:[1]

  • 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.[2]

DDx:

Images

  • Osteosarcoma - low mag. (WC)

  • Osteosarcoma - intermed. mag. (WC)

  • Osteosarcoma - high mag. (WC)

  • Osteosarcoma - very high mag. (WC)

  • Small cell osteosarcoma - intermed. mag. (WC)

  • Small cell osteosarcoma - high mag. (WC)

www:

Subtypes

Subtypes:[4][5]

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

Images:

Low-grade central osteosarcoma

  • Well-formed bone.
  • Usu. minimal nuclear atypia.

DDx:

Telangiectatic osteosarcoma

  • Rare.[7]
  • Extremely vascular.
  • Prognosis similar to other types of osteosarcoma.[8]

Important radiologic DDx:

Parosteal osteosarcoma

  • Arise from surface of bone.[9]
  • Low grade.[10]
  • Well-formed bone.

DDx:

Periosteal osteosarcoma

  • Intermediate grade.[10]

Small cell osteosarcoma

Secondary osteosarcoma

Arises in the context of something else - causes:

Images:

See also

References

  1. ↑ Greenwald, J.; Heng, M. (2007). Toronto Notes for Medical Students 2007 (2007 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. OR43. ISBN 978-0968592878.
  2. ↑ Papalas JA, Balmer NN, Wallace C, Sangueeza OP (June 2009). "Ossifying dermatofibroma with osteoclast-like giant cells: report of a case and literature review". Am J Dermatopathol 31 (4): 379-83. doi:10.1097/DAD.0b013e3181966747. PMID 19461244.
  3. ↑ Papandreou, C.; Skopelitou, A.; Kappes, G.; Daouaher, H. (2010). "Primary osteosarcoma of the urinary bladder treated with external radiotherapy in a patient with a history of transitional cell carcinoma: a case report.". J Med Case Rep 4: 70. doi:10.1186/1752-1947-4-70. PMC 2843711. PMID 20181254. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2843711/.
  4. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 638. ISBN 978-0781765275.
  5. ↑ URL: http://bestpractice.bmj.com/best-practice/monograph/780/basics/classification.html. Accessed on: 7 April 2011.
  6. ↑ Inwards, CY (2001). "Low-grade central osteosarcoma versus fibrous dysplasia". Pathology Case Reviews 6 (1): 22-27. http://journals.lww.com/pathologycasereviews/Fulltext/2001/01000/Low_Grade_Central_Osteosarcoma_Versus_Fibrous.5.aspx.
  7. ↑ Patibandla, MR.; Uppin, SG.; Thotakura, AK.; Panigrahi, MK.; Challa, S.. "Primary telangiectatic osteosarcoma of occipital bone: a case report and review of literature.". Neurol India 59 (1): 117-9. doi:10.4103/0028-3886.76891. PMID 21339678.
  8. ↑ Weiss, A.; Khoury, JD.; Hoffer, FA.; Wu, J.; Billups, CA.; Heck, RK.; Quintana, J.; Poe, D. et al. (Apr 2007). "Telangiectatic osteosarcoma: the St. Jude Children's Research Hospital's experience.". Cancer 109 (8): 1627-37. doi:10.1002/cncr.22574. PMID 17351949.
  9. ↑ The International Agency for Research on Cancer (Editors: Fletcher, C.D.M.; Unni, K. Krishnan; Mertens, F.) (2006). Pathology and Genetics of Tumours of Soft Tissue and Bone (IARC WHO Classification of Tumours) (3rd ed.). World Health Organization. pp. 279. ISBN 978-9283224136.
  10. ↑ 10.0 10.1 10.2 Carrle, D.; Bielack, SS. (Dec 2006). "Current strategies of chemotherapy in osteosarcoma.". Int Orthop 30 (6): 445-51. doi:10.1007/s00264-006-0192-x. PMC 3172747. PMID 16896870. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3172747/.
  11. ↑ URL: http://www.rsna.org/REG/publications/rg/afip/privateM/1997/0017/0005/1205/6.htm. Accessed on: 8 April 2011.
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