High-grade surface osteosarcoma
Updates to Article Attributes
High-grade surface osteosarcomas are very raresporadic and high-grade malignant osteoid forming-forming tumours emerging from the bony surfaces similar to parosteal and periosteal osteosarcomas.
Epidemiology
High-grade surface osteosarcomas are very rare and constitute <1% of all osteosarcomas. There seems to be a peak incidence in the 2nd and 3rd decade of life and a male predilectionpreference 1-5.
Diagnosis
The diagnosis of high-grade surface osteosarcomas is based on a combination of typical radiographic features with the histology of high-grade osteosarcoma.
Diagnostic criteria
Diagnostic criteria according to the WHO classification of soft tissue and bone tumours (2020 - blue book(5th edition) 1:
imaging features of a bone tumour
histological finding of a high-grade osteosarcoma
origin from the surface of the bone
no significant intraosseous part
Clinical presentation
The most common complaints are swelling and pain 1. On rare occasions, there is joint stiffness or even no symptoms at all 3,4.
Pathology
High-grade surface osteosarcomas are highly malignant osteosarcomas growing from the fibrous periosteal surfaces and are one of the three subtypes of surface osteosarcoma together with parosteal osteosarcoma and periosteal osteosarcoma 1-3.
Location
The tumour has been found in the following locations 1-5:
Macroscopic appearance
Macroscopically high-grade surface osteosarcomas are of variable appearance mostly dependantdependent on the type of generated extracellular matrix. The bulk of the tumour is usually located outside of the bone with infiltration and extension of the periosteum inwards and erosion of the underlying cortex 1.
Microscopic appearance
Microscopic features of high-grade surface osteosarcomas are those of a conventional high-grade osteosarcoma 1.
Immunophenotype
Irrelevant for diagnosis 1.
Radiographic features
General imaging features of high-grade surface osteosarcomas are the following 1,2,5:
the tumour arises from the bony surfaces
dense ossifications
cortical erosions (≈50% of the cases)
medullary involvement (≈50% of the cases)
usually no cleavage plane between the main tumour and cortex
periosteal reaction uncommon
relatively high circumferential involvement (20-100%)
Plain radiograph
On plain radiographs high-grade surface osteosarcomas will usually display the following characteristics 1:
radiodense ill-defined
fluffy immature ossification
CT
CT can demonstrate the following:
osteoid matrix
cortical erosions
cortical thickening
medullary involvement
MRI
MRI can accurately depict soft tissue and medullary involvement of high-grade surface osteosarcomas as well as peritumoral and/or medullary oedema.
-
T1
osteoid matrix: low signal intensity
non-ossified soft tissue components: intermediate signal intensity
-
T2
osteoid matrix: low signal intensity
non-ossified soft tissue components: high signal intensity
T1 C+ (Gd): marked enhancement
Radiology report
The radiological report should include a description of the following 1,6:
form, location and size
tumour margins and transition zone
circumferential extent
cortical erosion, cortical breakthrough
medullary components
soft tissue component
infiltration of neurovascular structures
Treatment and prognosis
Tumour management includes a combination of wide surgical excision and chemotherapy 2.
The five-year survival varies with different studies and ranges from roughly 40-80% 1-3. LocalizedLocalised disease and a good response to neoadjuvant good prognostic factors 1.
History and etymology
High-grade surface osteosarcoma was apparently first described by the American orthopaedic surgeon Kenneth C. Francis and his pathologist and surgeon colleagues Robert VP Hutter and Bradley L Coley in 1964 by Francis 2,3,7.
Differential diagnosis
Conditions that can mimic the presentation of high-grade surface osteosarcomas include 1,4:
-
usually aggressive periosteal reaction
less circumferential involvement
less medullary involvement
parosteal osteosarcoma: less circumferential involvement
osteosarcoma: the bulk of the tumour is located inside the bone
See also
-<p><strong>High-grade surface osteosarcomas</strong> are very rare and high-grade malignant osteoid forming tumours emerging from the bony surfaces similar to <a href="/articles/parosteal-osteosarcoma-1">parosteal</a> and <a href="/articles/periosteal-osteosarcoma">periosteal osteosarcomas</a>.</p><h4>Epidemiology</h4><p>High-grade surface osteosarcomas are very rare and constitute <1% of all osteosarcomas. There seems to be a peak incidence in the 2nd and 3rd decade of life and a male predilection <sup>1-5</sup>.</p><h4>Diagnosis</h4><p>The diagnosis of high-grade surface osteosarcomas is based on a combination of typical radiographic features with the histology of high-grade osteosarcoma.</p><h5>Diagnostic criteria</h5><p>Diagnostic criteria according to the <a href="/articles/who-classification-of-tumors-of-bone">WHO classification of bone tumours (2020 - blue book)</a> <sup>1</sup>:</p><ul>-<li>imaging features of a bone tumour</li>-<li>histological finding of a high-grade osteosarcoma</li>-<li>origin from the surface of the bone</li>-<li>no significant intraosseous part</li>- +<p><strong>High-grade surface osteosarcomas</strong> are sporadic and high-grade malignant osteoid-forming tumours emerging from the bony surfaces similar to <a href="/articles/parosteal-osteosarcoma-1">parosteal</a> and <a href="/articles/periosteal-osteosarcoma">periosteal osteosarcomas</a>.</p><h4>Epidemiology</h4><p>High-grade surface osteosarcomas are very rare and constitute <1% of all osteosarcomas. There seems to be a peak incidence in the 2nd and 3rd decade of life and a male preference <sup>1-5</sup>.</p><h4>Diagnosis</h4><p>The diagnosis of high-grade surface osteosarcomas is based on a combination of typical radiographic features with the histology of high-grade <a href="/articles/osteosarcoma" title="Osteosarcoma">osteosarcoma</a>.</p><h5>Diagnostic criteria</h5><p>Diagnostic criteria according to the <a href="/articles/who-classification-of-tumors-of-bone" title="WHO classification of bone tumours">WHO classification of soft tissue and bone tumours (5<sup>th</sup> edition)</a> <sup>1</sup>:</p><ul>
- +<li><p>imaging features of a bone tumour</p></li>
- +<li><p>histological finding of a high-grade osteosarcoma</p></li>
- +<li><p>origin from the surface of the bone</p></li>
- +<li><p>no significant intraosseous part</p></li>
-<li>diaphyseal and diametaphyseal areas of long bones</li>-<li>femur</li>-<li>tibia</li>-<li>humerus</li>-</ul><h5>Macroscopic appearance</h5><p>Macroscopically high-grade surface osteosarcomas are of variable appearance mostly dependant on the type of generated extracellular matrix. The bulk of the tumour is usually located outside of the bone with infiltration and extension of the periosteum inwards and erosion of the underlying cortex <sup>1</sup>.</p><h5>Microscopic appearance</h5><p>Microscopic features of high-grade surface osteosarcomas are those of a conventional high-grade <a href="/articles/osteosarcoma">osteosarcoma</a> <sup>1</sup>.</p><h5>Immunophenotype</h5><p>Irrelevant for diagnosis <sup>1</sup>.</p><h4>Radiographic features</h4><p>General imaging features of high-grade surface osteosarcomas are the following <sup>1,2,5</sup>:</p><ul>-<li>the tumour arises from the bony surfaces</li>-<li>dense ossifications</li>-<li>cortical erosions (≈50% of the cases)</li>-<li>medullary involvement (≈50% of the cases)</li>-<li>usually no cleavage plane between the main tumour and cortex</li>-<li>periosteal reaction uncommon</li>-<li>relatively high circumferential involvement (20-100%)</li>- +<li><p>diaphyseal and diametaphyseal areas of long bones</p></li>
- +<li><p><a href="/articles/femur" title="Femur">femur</a></p></li>
- +<li><p><a href="/articles/tibia" title="Tibia">tibia</a></p></li>
- +<li><p><a href="/articles/humerus" title="Humerus">humerus</a></p></li>
- +</ul><h5>Macroscopic appearance</h5><p>Macroscopically high-grade surface osteosarcomas are of variable appearance mostly dependent on the type of generated extracellular matrix. The bulk of the tumour is usually located outside of the bone with infiltration and extension of the periosteum inwards and erosion of the underlying cortex <sup>1</sup>.</p><h5>Microscopic appearance</h5><p>Microscopic features of high-grade surface osteosarcomas are those of conventional high-grade <a href="/articles/osteosarcoma">osteosarcoma</a> <sup>1</sup>.</p><h5>Immunophenotype</h5><p>Irrelevant for diagnosis <sup>1</sup>.</p><h4>Radiographic features</h4><p>General imaging features of high-grade surface osteosarcomas are the following <sup>1,2,5</sup>:</p><ul>
- +<li><p>the tumour arises from the bony surfaces</p></li>
- +<li><p>dense ossifications</p></li>
- +<li><p>cortical erosions (≈50% of the cases)</p></li>
- +<li><p>medullary involvement (≈50% of the cases)</p></li>
- +<li><p>usually no cleavage plane between the main tumour and cortex</p></li>
- +<li><p>periosteal reaction uncommon</p></li>
- +<li><p>relatively high circumferential involvement (20-100%)</p></li>
-<li>radiodense ill-defined</li>-<li>fluffy immature ossification</li>- +<li><p>radiodense ill-defined</p></li>
- +<li><p>fluffy immature ossification</p></li>
-<li>osteoid matrix</li>-<li>cortical erosions</li>-<li>cortical thickening</li>-<li>medullary involvement</li>- +<li><p>osteoid matrix</p></li>
- +<li><p>cortical erosions</p></li>
- +<li><p>cortical thickening</p></li>
- +<li><p>medullary involvement</p></li>
-<strong>T1</strong><ul>-<li>osteoid matrix: low signal intensity</li>-<li>non-ossified soft tissue components: intermediate signal intensity</li>- +<p><strong>T1</strong></p>
- +<ul>
- +<li><p>osteoid matrix: low signal intensity</p></li>
- +<li><p>non-ossified soft tissue components: intermediate signal intensity</p></li>
-<strong>T2</strong><ul>-<li>osteoid matrix: low signal intensity</li>-<li>non-ossified soft tissue components: high signal intensity</li>- +<p><strong>T2</strong></p>
- +<ul>
- +<li><p>osteoid matrix: low signal intensity</p></li>
- +<li><p>non-ossified soft tissue components: high signal intensity</p></li>
-<li>-<strong>T1 C+ (Gd): </strong>marked enhancement</li>- +<li><p><strong>T1 C+ (Gd): </strong>marked enhancement</p></li>
-<li>form, location and size</li>-<li>tumour margins and transition zone</li>-<li>circumferential extent</li>-<li>cortical erosion, cortical breakthrough</li>-<li>medullary components</li>-<li>soft tissue component</li>-<li>infiltration of neurovascular structures</li>-</ul><h4>Treatment and prognosis</h4><p>Tumour management includes a combination of wide surgical excision and chemotherapy <sup>2</sup>.</p><p>The five-year survival varies with different studies and ranges from roughly 40-80% <sup>1-3</sup>. Localized disease and a good response to neoadjuvant good prognostic factors <sup>1</sup>.</p><h4>History and etymology</h4><p>High-grade surface osteosarcoma was apparently first described in 1964 by Francis <sup>2,3</sup>.</p><h4>Differential diagnosis</h4><p>Conditions that can mimic the presentation of high-grade surface osteosarcomas include <sup>1,4</sup>:</p><ul>-<li>-<a href="/articles/periosteal-osteosarcoma">periosteal osteosarcoma</a><ul>-<li>usually aggressive periosteal reaction</li>-<li>less circumferential involvement</li>-<li>less medullary involvement</li>- +<li><p>form, location and size</p></li>
- +<li><p>tumour margins and transition zone</p></li>
- +<li><p>circumferential extent</p></li>
- +<li><p>cortical erosion, cortical breakthrough</p></li>
- +<li><p>medullary components</p></li>
- +<li><p>soft tissue component</p></li>
- +<li><p>infiltration of neurovascular structures</p></li>
- +</ul><h4>Treatment and prognosis</h4><p>Tumour management includes a combination of wide surgical excision and chemotherapy <sup>2</sup>.</p><p>The five-year survival varies with different studies and ranges from roughly 40-80% <sup>1-3</sup>. Localised disease and a good response to neoadjuvant good prognostic factors <sup>1</sup>.</p><h4>History and etymology</h4><p>High-grade surface osteosarcoma was first described by the American orthopaedic surgeon <strong>Kenneth C. Francis</strong> and his pathologist and surgeon colleagues <strong>Robert VP Hutter</strong> and <strong>Bradley L Coley</strong> in 1964 <sup>2,3,7</sup>.</p><h4>Differential diagnosis</h4><p>Conditions that can mimic the presentation of high-grade surface osteosarcomas include <sup>1,4</sup>:</p><ul>
- +<li>
- +<p><a href="/articles/periosteal-osteosarcoma">periosteal osteosarcoma</a></p>
- +<ul>
- +<li><p>usually aggressive periosteal reaction</p></li>
- +<li><p>less circumferential involvement</p></li>
- +<li><p>less medullary involvement</p></li>
-<li>-<a href="/articles/parosteal-osteosarcoma-1">parosteal osteosarcoma</a>: less circumferential involvement</li>-<li>-<a href="/articles/osteosarcoma">osteosarcoma</a>: the bulk of the tumour is located inside the bone</li>-<li><a href="/articles/myositis-ossificans-1">myositis ossificans</a></li>-</ul><h4>See also</h4><ul><li><a href="/articles/osteosarcoma">osteosarcoma</a></li></ul>- +<li><p><a href="/articles/parosteal-osteosarcoma-1">parosteal osteosarcoma</a>: less circumferential involvement</p></li>
- +<li><p><a href="/articles/osteosarcoma">osteosarcoma</a>: the bulk of the tumour is located inside the bone</p></li>
- +<li><p><a href="/articles/myositis-ossificans-1">myositis ossificans</a></p></li>
- +</ul>
References changed:
- 1. Klein MJ, Bonar SFM, O’Donell PG. High-grade surface osteosarcoma. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <a href="https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Soft-Tissue-And-Bone-Tumours-2020">https://publications.iarc.fr</a>
- 2. Staals E, Bacchini P, Bertoni F. High-Grade Surface Osteosarcoma. Cancer. 2008;112(7):1592-9. <a href="https://doi.org/10.1002/cncr.23340">doi:10.1002/cncr.23340</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18300258">Pubmed</a>
- 3. Deng Z, Huang Z, Ding Y, Su Y, Chan C, Niu X. High-Grade Surface Osteosarcoma: Clinical Features and Oncologic Outcome. Journal of Bone Oncology. 2020;23:100288. <a href="https://doi.org/10.1016/j.jbo.2020.100288">doi:10.1016/j.jbo.2020.100288</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32953434">Pubmed</a>
- 4. Nouri H, Ben Maitigue M, Abid L et al. Surface Osteosarcoma: Clinical Features and Therapeutic Implications. Journal of Bone Oncology. 2015;4(4):115-23. <a href="https://doi.org/10.1016/j.jbo.2015.07.002">doi:10.1016/j.jbo.2015.07.002</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26730360">Pubmed</a>
- 5. Yarmish G, Klein M, Landa J, Lefkowitz R, Hwang S. Imaging Characteristics of Primary Osteosarcoma: Nonconventional Subtypes. Radiographics. 2010;30(6):1653-72. <a href="https://doi.org/10.1148/rg.306105524">doi:10.1148/rg.306105524</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/21071381">Pubmed</a>
- 7. Francis KC,Hutter RVP,Coley BL. Treatment of osteogenic sarcoma. In: GT Pack, IM Ariel, editors. Treatment of Cancer and Allied Diseases. Vol. 8, 2nd ed. New York: Harper & Row; 1964: 374– 399.
- 1. W. H. O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020) ISBN: 9789283245025
- 2. Staals E, Bacchini P, Bertoni F. High-Grade Surface Osteosarcoma. Cancer. 2008;112(7):1592-9. <a href="https://doi.org/10.1002/cncr.23340">doi:10.1002/cncr.23340</a>
- 3. Deng Z, Huang Z, Ding Y, Su Y, Chan C, Niu X. High-Grade Surface Osteosarcoma: Clinical Features and Oncologic Outcome. Journal of Bone Oncology. 2020;23:100288. <a href="https://doi.org/10.1016/j.jbo.2020.100288">doi:10.1016/j.jbo.2020.100288</a>
- 4. Nouri H, Ben Maitigue M, Abid L et al. Surface Osteosarcoma: Clinical Features and Therapeutic Implications. Journal of Bone Oncology. 2015;4(4):115-23. <a href="https://doi.org/10.1016/j.jbo.2015.07.002">doi:10.1016/j.jbo.2015.07.002</a>
- 5. Yarmish G, Klein M, Landa J, Lefkowitz R, Hwang S. Imaging Characteristics of Primary Osteosarcoma: Nonconventional Subtypes. RadioGraphics. 2010;30(6):1653-72. <a href="https://doi.org/10.1148/rg.306105524">doi:10.1148/rg.306105524</a>
Tags changed:
- bone tumour
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