High-grade surface osteosarcoma
Updates to Article Attributes
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 22nd and nd33rd decade of life and a male predilection 1-5.rd
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:
-
periosteal osteosarcoma
- 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
- myositis ossificans
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 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 <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:
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