Telangiectatic osteosarcoma
Updates to Article Attributes
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was changed:
Telanagiectatic osteosarcomas (TOS) is are an uncommon variant of osteosarcoma. It is thought to represent that represent between 2.5 -and 12 %percent of all osteosarcomas 6.
Epidemiology
Has similar demographics to that of conventional osteosarcoma and typically presents in adolescents and young adults (reported age range of 3 - 67-67 years with a mean age of 20 years). There is a recognised male predilection.
Pathology
Most osteosarcomas have a small telangiectatic component but in order to classify as a telangiectatic osteosarcoma the telangiectatic component should comprise more than 90% 1. Most of the tumour comprises of large blood filled spaces separated by thin bony septations.
Microscopically the tumour consist of vascular sinusoids surrounded by thin septae, osteoid matrix and cells with significant pleomorphism and high mitotic rate.
Associations
A telangiectatic osteosarcoma can be a secondary lesion (arising in association with fibrous dysplasia, Paget disease, or post radiation therapy).
Location
In terms of site a most common locations are:
- around knee: femur (
~ 50 - 62~50-62%) / tibia (~ 10 - 25~10-25%) - humerus:
~ 12 - 16~12-16%
In terms of location within bone:
- metaphyseal:
~ 80~80% - diaphyseal:
~ 20~20%
Radiographic features
Plain film
Typically seen as an expansatileexpansile lytic metaphyseal bony lesion.
- wide zone of transition
- geographic bony destruction tends to be more common than permeative bony destruction 6
- pathological fractures are frequent
CT
- low attenuating fluid-fluid levels within the lesion (in
~ 80~80% of cases). Useful -
useful at assessing associated cortical destruction
. -
thickthick peripheral and nodular septal enhancement. -
osteoidosteoid matrixmineralization.mineralisation
MRI
- commonly shows fluid-fluid levels within the lesion (
~ 90~90% of cases) with differing signal intensity. Allows -
allows appreciation of surrounding soft tissue components
. -
Signalsignal characteristics are often heterogeneous. Enhancement -
enhancement of septations as well as the soft tissue component may be observed
. - haemorrhage appears as
hperintensehyperintense on T1 and variable signal intensity on T2.
Bone scintigraphy
Overall lesion, lesions tends to show marked but heterogenousheterogeneous uptake. May demonstrate a region of central photopenia representing a doughnut sign 3,6.
Complications
They are associated with a high rate of pathological fractures 4.
Treatment and prognosis
The treatment of telangiectatic osteosarcoma is often similar to that of conventional osteosarcoma : chemotherapy followed by wide surgical resection and limb salvage or amputation.
Survival rate of telangiectatic osteosarcoma (estimated at ~ 68 ~68%) is similar to that of conventional osteosarcoma.
Etymology
First described by J Paget in 1854 7
Differential diagnosis
Conisderations on plain film include
- aneurysmal bone cyst (ABC): should not have any soft tissue component outside the lesion
-
giant cell tumour of bone:
againagain no soft tissue component - osteolytic metastatic bone lesions: should not have any fluid levels
.
Etymology
First described by J Paget in 1854 7.
-<p>A <strong>telenagiectatic osteosarcoma (TOS)</strong> is an uncommon variant of <a href="/articles/osteosarcoma">osteosarcoma</a>. It is thought to represent between 2.5 - 12 % of all osteosarcomas <sup>6</sup>. </p><h4>Epidemiology</h4><p>Has similar demographics to that of conventional osteosarcoma and typically presents in adolescents and young adults (reported age range of 3 - 67 years with a mean age of 20 years). There is a recognised male predilection.</p><h4>Pathology</h4><p>Most osteosarcomas have a small telangiectatic component but in order to classify as a telangiectatic osteosarcoma the telangiectatic component should comprise more than 90% <sup>1</sup>. Most of the tumour comprises of large blood filled spaces separated by thin bony septations.</p><p>Microscopically the tumour consist of vascular sinusoids surrounded by thin septae, osteoid matrix and cells with significant pleomorphism and high mitotic rate.</p><h5>Associations</h5><p>A telangiectatic osteosarcoma can be a secondary lesion (arising in association with <a href="/articles/fibrous-dysplasia">fibrous dysplasia</a>, <a href="/articles/pagets-disease-of-bone-1">Paget disease</a>, or post radiation therapy).</p><h5>Location </h5><p>In terms of site a most common locations are</p><ul>-<li>around knee : femur (~ 50 - 62 %) / tibia ( ~ 10 - 25 %)</li>-<li>humerus : ~ 12 - 16 %</li>-</ul><p>In terms of location within bone</p><ul>-<li>metaphyseal : ~ 80 %</li>-<li>diaphyseal : ~ 20 % </li>-</ul><h4>Radiographic features</h4><h5>Plain film</h5><p>Typically seen as an expansatile lytic metaphyseal bony lesion. </p><ul>- +<p><strong>Telanagiectatic osteosarcomas (TOS)</strong> are an uncommon variant of <a href="/articles/osteosarcoma">osteosarcoma</a> that represent between 2.5 and 12 percent of all osteosarcomas <sup>6</sup>. </p><h4>Epidemiology</h4><p>Has similar demographics to that of conventional osteosarcoma and typically presents in adolescents and young adults (reported age range of 3-67 years with a mean age of 20 years). There is a recognised male predilection.</p><h4>Pathology</h4><p>Most osteosarcomas have a small telangiectatic component but in order to classify as a telangiectatic osteosarcoma the telangiectatic component should comprise more than 90% <sup>1</sup>. Most of the tumour comprises of large blood filled spaces separated by thin bony septations.</p><p>Microscopically the tumour consist of vascular sinusoids surrounded by thin septae, osteoid matrix and cells with significant pleomorphism and high mitotic rate.</p><h5>Associations</h5><p>A telangiectatic osteosarcoma can be a secondary lesion (arising in association with <a href="/articles/fibrous-dysplasia">fibrous dysplasia</a>, <a href="/articles/pagets-disease-of-bone-1">Paget disease</a>, or post radiation therapy).</p><h5>Location </h5><p>In terms of site a most common locations are:</p><ul>
- +<li>around knee: femur (~50-62%) / tibia (~10-25%)</li>
- +<li>humerus: ~12-16%</li>
- +</ul><p>In terms of location within bone:</p><ul>
- +<li>metaphyseal: ~80%</li>
- +<li>diaphyseal: ~20% </li>
- +</ul><h4>Radiographic features</h4><h5>Plain film</h5><p>Typically seen as an expansile lytic metaphyseal bony lesion. </p><ul>
-<li>low attenuating <a href="/articles/fluid-fluid-level-containing-bone-lesions">fluid-fluid levels</a> within the lesion (in ~ 80 % of cases). Useful at assessing associated cortical destruction.</li>-<li> thick peripheral and nodular septal enhancement.</li>-<li> osteoid matrix mineralization. </li>- +<li>low attenuating <a href="/articles/fluid-fluid-level-containing-bone-lesions">fluid-fluid levels</a> within the lesion (in ~80% of cases)</li>
- +<li>useful at assessing associated cortical destruction</li>
- +<li>thick peripheral and nodular septal enhancement</li>
- +<li>osteoid matrix mineralisation</li>
-<li>commonly shows <a href="/articles/fluid-fluid-level-containing-bone-lesions">fluid-fluid levels</a> within the lesion ( ~ 90 % of cases ) with differing signal intensity. Allows appreciation of surrounding soft tissue components.</li>-<li>Signal characteristics are often heterogeneous. Enhancement of septations as well as the soft tissue component may be observed. </li>-<li>haemorrhage appears as hperintense on T1 and variable signal intensity on T2.</li>-</ul><h5>Bone scintigraphy</h5><p>Overall lesion tends to show marked but heterogenous uptake. May demonstrate a region of central photopenia representing a doughnut sign <sup>3,6</sup>.</p><h4>Complications</h4><p>They are associated with a high rate of <a href="/articles/pathological-fracture">pathological fractures</a> <sup>4</sup>.</p><h4>Treatment and prognosis</h4><p>The treatment of telangiectatic osteosarcoma is often similar to that of conventional osteosarcoma : chemotherapy followed by wide surgical resection and limb salvage or amputation. </p><p>Survival rate of telangiectatic osteosarcoma (estimated at ~ 68 %) is similar to that of conventional osteosarcoma.</p><h4>Etymology</h4><p>First described by <strong>J Paget</strong> in 1854 <sup>7</sup></p><h4>Differential diagnosis</h4><h6>Conisderations on plain film include</h6><ul>- +<li>commonly shows <a href="/articles/fluid-fluid-level-containing-bone-lesions">fluid-fluid levels</a> within the lesion ( ~90% of cases) with differing signal intensity</li>
- +<li>allows appreciation of surrounding soft tissue components</li>
- +<li>signal characteristics are often heterogeneous</li>
- +<li>enhancement of septations as well as the soft tissue component may be observed</li>
- +<li>haemorrhage appears as hyperintense on T1 and variable signal intensity on T2</li>
- +</ul><h5>Bone scintigraphy</h5><p>Overall, lesions tends to show marked but heterogeneous uptake. May demonstrate a region of central photopenia representing a doughnut sign <sup>3,6</sup>.</p><h4>Complications</h4><p>They are associated with a high rate of <a href="/articles/pathological-fracture">pathological fractures</a> <sup>4</sup>.</p><h4>Treatment and prognosis</h4><p>The treatment of telangiectatic osteosarcoma is often similar to that of conventional osteosarcoma : chemotherapy followed by wide surgical resection and limb salvage or amputation. </p><p>Survival rate of telangiectatic osteosarcoma (estimated at ~68%) is similar to that of conventional osteosarcoma.</p><h4>Differential diagnosis</h4><h6>Conisderations on plain film include</h6><ul>
-<a href="/articles/aneurysmal-bone-cyst">aneurysmal bone cyst (ABC)</a> : should not have any soft tissue component outside the lesion</li>- +<a href="/articles/aneurysmal-bone-cyst">aneurysmal bone cyst (ABC)</a>: should not have any soft tissue component outside the lesion</li>
-<a href="/articles/giant-cell-tumour-of-bone">giant cell tumour of bone</a> : again no soft tissue component </li>-<li>osteolytic <a href="/articles/mixed-lytic-and-sclerotic-bone-metastases">metastatic bone lesions</a>: should not have any fluid levels.</li>-</ul>- +<a href="/articles/giant-cell-tumour-of-bone">giant cell tumour of bone</a>: again no soft tissue component </li>
- +<li>osteolytic <a href="/articles/mixed-lytic-and-sclerotic-bone-metastases">metastatic bone lesions</a>: should not have any fluid levels</li>
- +</ul><h4>Etymology</h4><p>First described by <strong>J Paget</strong> in 1854 <sup>7</sup>.</p>
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