High-grade surface osteosarcoma

Changed by Joachim Feger, 4 Sep 2021

Updates to Article Attributes

Body was changed:

High-grade surface osteosarcomas are very rare and high-grade malignant osteoid 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 2nd2nd and 3rd3rd decade of life and a male predilection 1-5.

Clinical presentation

MostThe 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:

  • diaphyseal and diametaphyseal areas of long bones
  • femur
  • tibia
  • humerus
Macroscopic appearance

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 1.

Microscopic appearance

Microscopic features of high-grade surface osteosarcomas are those of a conventional high-grade osteosarcoma 1.

ImmunohistochemistryImmunophenotype

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≈50% of the cases)
  • medullary involvement (≈ 50≈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 is able tocan 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. Localized disease and a good response to neoadjuvant good prognostic factors 1.

History and etymology

High-grade surface osteosarcoma was apparently first described in 1964 by Francis 2,3.

Differential diagnosis

Conditions whichthat can mimic the presentation of high-grade surface osteosarcomas include 1,4:

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 &lt;1% of all osteosarcomas. There seems to be a peak incidence in the 2<sup>nd</sup> and 3<sup>rd</sup> decade of life and a male predilection <sup>1-5</sup>.</p><h4>Clinical presentation</h4><p>Most common complaints are swelling and pain <sup>1</sup>. On rare occasions, there is joint stiffness or even no symptoms at all <sup>3,4</sup>.</p><h4>Pathology</h4><p>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 <a href="/articles/parosteal-osteosarcoma-1">parosteal osteosarcoma</a> and <a href="/articles/periosteal-osteosarcoma">periosteal osteosarcoma</a> <sup>1-3</sup>.</p><h5>Location</h5><p>The tumour has been found in the following locations <sup>1-5</sup><strong>:</strong></p><ul>
  • +<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 &lt;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>Clinical presentation</h4><p>The most common complaints are swelling and pain <sup>1</sup>. On rare occasions, there is joint stiffness or even no symptoms at all <sup>3,4</sup>.</p><h4>Pathology</h4><p>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 <a href="/articles/parosteal-osteosarcoma-1">parosteal osteosarcoma</a> and <a href="/articles/periosteal-osteosarcoma">periosteal osteosarcoma</a> <sup>1-3</sup>.</p><h5>Location</h5><p>The tumour has been found in the following locations <sup>1-5</sup><strong>:</strong></p><ul>
  • -</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>Immunohistochemistry</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>
  • +</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>cortical erosions (≈ 50% of the cases)</li>
  • -<li>medullary involvement (≈ 50% of the cases)</li>
  • -<li>usually no cleavage plane between main tumour and cortex</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>
  • -</ul><h5>MRI</h5><p>MRI is able to accurately depict soft tissue and medullary involvement of high-grade surface osteosarcomas as well as peritumoral and/or medullary oedema.</p><ul>
  • +</ul><h5>MRI</h5><p>MRI can accurately depict soft tissue and medullary involvement of high-grade surface osteosarcomas as well as peritumoral and/or medullary oedema.</p><ul>
  • -</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 which can mimic the presentation of high-grade surface osteosarcomas include <sup>1,4</sup>:</p><ul>
  • +</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>

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>
  • 1. W.H.O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020) <a href="https://books.google.co.uk/books?vid=ISBN9789283245025">ISBN: 9789283245025</a><span class="ref_v4"></span>
  • 2. Staals EL, Bacchini P, Bertoni F. High-grade surface osteosarcoma: a review of 25 cases from the Rizzoli Institute. (2008) Cancer. 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> <span class="ref_v4"></span>
  • 3. Deng Z, Huang Z, Ding Y, Su Y, Chan CM, Niu X. High-Grade Surface Osteosarcoma: Clinical Features and Oncologic Outcome. (2020) Journal of bone oncology. 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> <span class="ref_v4"></span>
  • 4. Nouri H, Ben Maitigue M, Abid L, Nouri N, Abdelkader A, Bouaziz M, Mestiri M. Surface osteosarcoma: Clinical features and therapeutic implications. (2015) Journal of bone oncology. 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> <span class="ref_v4"></span>
  • 5. Yarmish G, Klein MJ, Landa J, Lefkowitz RA, Hwang S. Imaging characteristics of primary osteosarcoma: nonconventional subtypes. (2010) Radiographics : a review publication of the Radiological Society of North America, Inc. 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> <span class="ref_v4"></span>

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.