Chondrosarcoma

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Chondrosarcomas are malignant cartilaginous tumours that account for ~~20-27~~~25% of all primary malignant bone tumours. ~~They~~The are most commonly found in long bones ~~due a more abundant cartilage presence~~, and ~~are diagnosed in patients in their 4^th ~~and 5~~^th ~~decades of life. There are two main subtypes: primary chondrosarcomas (de~~~~can arise de novo ~~lesions) and~~ or secondary ~~chondrosarcomas (superimposed on preexisting~~to an existing benign cartilaginous neoplasms).

On On imaging these tumours have ~~suggestive radiographic~~ring-and-arc chondroid matrix mineralisation with aggressive features such as lytic pattern, ~~typical ring-and-arc chondroid matrix mineralization, deep~~ deep endosteal scalloping and soft-tissue extension. CT additionally depicts the matrix mineralization. The high water content of these lesions are expressed as low attenuation on CT and very high signal intensity with T2-weighted MRI sequences. Contrast enhancement is heterogeneous and when more prominent and diffuse it frequently suggests more aggressive types of chondrosarcomas.

Epidemiology

Typical presentation is in the 4^th and 5^th decades and there is a slight male predominance of 1.5-2:1.

Clinical presentation

Patients usually present with pain, pathological fracture, a palpable lump or local mass effect. Hyperglycaemia can occur as a paraneoplastic syndrome.

Pathology

The histology of chondrosarcomas can differ according to their sub type (see below). In general these tumours are multilobulated (due to hyaline cartilage nodules) with central high water content and peripheral enchondral ossification. This accounts not only for the high T2 MRI signal (see below) but also forrings and arcs calcification or popcorn calcification.

Grading

Chondrosarcomas are divided into 3three (sometimes 4four) grades based primarily on cellularity (see: chondrosarcoma grading).

Subtypes

Chondrosarcomas are either primary, arising de novo, or secondary and arise from a pre-existent ~~cartilagenous~~cartilaginous mass (see: ~~see~~ secondary chondrosarcoma).

Primary

conventional intramedullary chondrosarcoma (or central chondrosarcoma): low, intermediate or high grade (see: chondrosarcoma grading)
juxtacortical chondrosarcoma: low, intermediate or high grade
clear cell chondrosarcoma
myxoid chondrosarcoma: usually intermediate grade
mesenchymal chondrosarcoma: usually high grade
extraskeletal chondrosarcoma
dedifferentiated chondrosarcoma

Secondary

Arising from pre-existing cartilagenous lesions:

osteochondroma
- solitary osteochondroma
- hereditary multiple exostoses ~~(also known as diaphyseal aclasis)~~
enchondroma
- solitary enchondroma
- Ollier disease
- Maffucci syndrome

Distribution

long bones: 45% (the reason is because the cartilage is more abundant in the long, tubular bones).
- femur: 20-35%
- tibia: 5%
- upper limb (especially proximal humerus): 10-20%

pelvis: 25% especially around the triradiate cartilage
ribs: 8%
- patients often younger than at other sites
- anterior ribs/costochondral junction
spine: 7%
- greater male predominance 2-4:1
- thoracic most common
- location
  - posterior elements and vertebral body 45%
  - posterior elements only 40%
  - vertebral body only 15%
scapula: 5%
sternum: 2%
head and neck (including cervical spine): 6-7%
craniofacial: 2% (see chondrdosarcoma of the skull of base)
hands and feet: rare c.f. enchondromas

Radiographic features

Imaging findings vary somewhat with different sub types but do have some general features. Below are typical imaging appearances which are best demonstrated by conventional chondrosarcomas.

In general chondrosarcomas are large masses at the time of diagnosis, usually ~~over 4~~>4 cm in diameter and ~~over 10~~>10 cm in 50% of cases.

RadiographPlain radiograph

lytic (50%)
intralesional ~~calcification(s)~~calcifications: ~~60-78~~~70% (rings and arcs calcification or popcorn calcification)
endosteal scalloping: affecting more than two thirds of the cortical thickness (c.f. less than ~~2/3~~two-thirds in enchondromas)
moth eaten appearance or permeative appearance in higher grade tumours (see chondrosarcoma grading), e.g. myxoid, dedifferentiated and mesenchymal chondrosarcomas
cortical remodelling, thickening and periosteal reaction are also useful in distinguishing between an enchondroma and low grade chondrosarcoma (seeenchondroma vs. low grade chondrosarcoma)

CT

The features seen on CT are the same as on plain film, but are simply better seen.

94% of cases demonstrate matrix calcificaiton, c.f. 60-78% on plain film.
endosteal scalloping
cortical breach, seen in 88~90% of ~~longbone~~long bone chondrosarcoma, c.f. only 8~10% of enchondromas
soft tissue mass: density increases with increased grade of tumour do to increased cellularity
heterogenous contrast enhancment

MRI

T1: low to intermediate signal
- iso- to slightly hyperintense cf. muscle
- iso- to slightly hypointense cf. grey matter (see chondrosarcoma of the base of skull)
T2: very high intensity in nonmineralised/calcified portions
gradient echo/SWI: blooming of mineralised/calcified portions
T1 C+ (Gd)
- most demonstrate heterogeneous moderate to intense contrast enhancement.
- enhancement can be septal and peripheral rim-like corresponding to fibrovascular septation between lobules of hyaline cartilage

Nuclear medicine

Typically chondrosarcomas demonstrate increased uptake onbone scan, seen in over 80% of cases, and usually the uptake is quite intense. This is useful in helping to distinguish a low grade chondrosarcoma from an enchondroma as the ~~later~~latter has increased uptake in ~~only 21~~~20% of cases, and usually to a lesser degree. (see: enchondroma vs. low grade chondrosarcoma).

Treatment and prognosis

Prognosis varies with both grade and location. In general:

grade
- grade 1: 90% 5 ~~year~~-year survival
- grade 3: 29% 5 ~~year~~-year survival
location
- long bones have a better prognosis than axial skeleton

-<p><strong>Chondrosarcomas</strong> are malignant cartilaginous tumours that account for 20-27% of all <a href="/articles/primary-malignant-bone-tumours">primary malignant bone tumours</a>. They are commonly found in long bones due a more abundant cartilage presence, and are diagnosed in patients in their 4<sup>th</sup> and 5<sup>th</sup> decades of life. There are two main subtypes: primary chondrosarcomas (de novo lesions) and secondary chondrosarcomas (superimposed on preexisting benign cartilaginous neoplasms).</p><p>On imaging these tumours have suggestive radiographic features such as lytic pattern, typical ring-and-arc chondroid matrix mineralization, deep endosteal scalloping and soft-tissue extension. CT additionally depicts the matrix mineralization. The high water content of these lesions are expressed as low attenuation on CT and very high signal intensity with T2-weighted MRI sequences. Contrast enhancement is heterogeneous and when more prominent and diffuse it frequently suggests more aggressive types of chondrosarcomas.</p><h4>Epidemiology</h4><p>Typical presentation is in the 4<sup>th</sup> and 5<sup>th</sup> decades and there is a slight male predominance of 1.5-2:1.</p><h4>Clinical presentation</h4><p>Patients usually present with pain, pathological fracture, a palpable lump or local mass effect. <a href="/articles/hyperglycaemia">Hyperglycaemia</a> can occur as a <a href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a>.</p><h4>Pathology</h4><p>The histology of chondrosarcomas can differ according to their sub type (see below). In general these tumours are multilobulated (due to hyaline cartilage nodules) with central high water content and peripheral enchondral ossification. This accounts not only for the high T2 MRI signal (see below) but also for <a href="/articles/rings-and-arcs-calcification">rings and arcs calcification</a> or <a href="/articles/popcorn-calcification">popcorn calcification</a>. </p><h5>Grading</h5><p>Chondrosarcomas are divided into 3 (sometimes 4) grades based primarily on cellularity (see <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>).</p><h5>Subtypes</h5><p>Chondrosarcomas are either primary, arising de novo, or secondary and arise from a pre-existent cartilagenous mass: see <a href="/articles/secondary-chondrosarcoma">secondary chondrosarcoma</a>.</p><h6>Primary</h6><ul>
+<p><strong>Chondrosarcomas</strong> are malignant cartilaginous tumours that account for ~25% of all <a href="/articles/primary-malignant-bone-tumours">primary malignant bone tumours</a>. The are most commonly found in long bones, and can arise <em>de novo</em> or secondary to an existing benign cartilaginous neoplasms). On imaging these tumours have ring-and-arc chondroid matrix mineralisation with aggressive features such as lytic pattern, deep endosteal scalloping and soft-tissue extension. </p><h4>Epidemiology</h4><p>Typical presentation is in the 4<sup>th</sup> and 5<sup>th</sup> decades and there is a slight male predominance of 1.5-2:1.</p><h4>Clinical presentation</h4><p>Patients usually present with pain, pathological fracture, a palpable lump or local mass effect. <a href="/articles/hyperglycaemia">Hyperglycaemia</a> can occur as a <a href="/articles/paraneoplastic-syndromes">paraneoplastic syndrome</a>.</p><h4>Pathology</h4><p>The histology of chondrosarcomas can differ according to their sub type (see below). In general these tumours are multilobulated (due to hyaline cartilage nodules) with central high water content and peripheral enchondral ossification. This accounts not only for the high T2 MRI signal (see below) but also for <a href="/articles/rings-and-arcs-calcification">rings and arcs calcification</a> or <a href="/articles/popcorn-calcification">popcorn calcification</a>. </p><h5>Grading</h5><p>Chondrosarcomas are divided into three (sometimes four) grades based primarily on cellularity (see: <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>).</p><h5>Subtypes</h5><p>Chondrosarcomas are either primary, arising <em>de novo</em>, or secondary and arise from a pre-existent cartilaginous mass (see: <a href="/articles/secondary-chondrosarcoma">secondary chondrosarcoma</a>).</p><h6>Primary</h6><ul>
-<a href="/articles/conventional-intramedullary-chondrosarcoma">conventional intramedullary chondrosarcoma</a> (or central chondrosarcoma): low, intermediate or high grade (see <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>)</li>
+<a href="/articles/conventional-intramedullary-chondrosarcoma">conventional intramedullary chondrosarcoma</a> (or central chondrosarcoma): low, intermediate or high grade (see: <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>)</li>
~~-<li>~~
~~-<a href="/articles/hereditary-multiple-exostoses">hereditary multiple exostoses</a> (also known as diaphyseal aclasis)</li>~~
+<li><a href="/articles/hereditary-multiple-exostoses">hereditary multiple exostoses</a></li>
~~-</ul><h5>Distribution</h5><ul><li>long bones: 45% (the reason is because the cartilage is more abundant in the long, tubular bones).<ul>~~
+</ul><h5>Distribution</h5><ul><li>long bones: 45% (the reason is because the cartilage is more abundant in the long, tubular bones)<ul>
-</ul><h4>Radiographic features</h4><p>Imaging findings vary somewhat with different sub types but do have some general features. Below are typical imaging appearances which are best demonstrated by conventional chondrosarcomas.</p><p>In general chondrosarcomas are large masses at the time of diagnosis, usually over 4 cm in diameter and over 10 cm in 50% of cases.</p><h5>Radiograph</h5><ul>
+</ul><h4>Radiographic features</h4><p>Imaging findings vary somewhat with different sub types but do have some general features. Below are typical imaging appearances which are best demonstrated by conventional chondrosarcomas.</p><p>In general chondrosarcomas are large masses at the time of diagnosis, usually >4 cm in diameter and >10 cm in 50% of cases.</p><h5>Plain radiograph</h5><ul>
-<li>intralesional calcification(s): 60-78% (<a href="/articles/rings-and-arcs-calcification">rings and arcs calcification</a> or <a href="/articles/popcorn-calcification">popcorn calcification</a>)</li>
+<li>intralesional calcifications: ~70% (<a href="/articles/rings-and-arcs-calcification">rings and arcs calcification</a> or <a href="/articles/popcorn-calcification">popcorn calcification</a>)</li>
~~-<a href="/articles/endosteal-scalloping">endosteal scalloping</a>: affecting more than two thirds of the cortical thickness (c.f. less than 2/3 in enchondromas) </li>~~
-<li>moth eaten appearance or <a href="/articles/permeative-process-in-bone">permeative appearance</a> in higher grade tumours (see <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>), e.g <a href="/articles/myxoid-chondrosarcoma">myxoid</a>, <a href="/articles/dedifferentiated-chondrosarcoma">dedifferentiated</a> and <a href="/articles/mesenchymal-chondrosarcoma">mesenchymal chondrosarcomas</a>
+<a href="/articles/endosteal-scalloping">endosteal scalloping</a>: affecting more than two thirds of the cortical thickness (c.f. less than two-thirds in enchondromas) </li>
+<li>moth eaten appearance or <a href="/articles/permeative-process-in-bone">permeative appearance</a> in higher grade tumours (see <a href="/articles/chondrosarcoma-grading">chondrosarcoma grading</a>), e.g. <a href="/articles/myxoid-chondrosarcoma">myxoid</a>, <a href="/articles/dedifferentiated-chondrosarcoma">dedifferentiated</a> and <a href="/articles/mesenchymal-chondrosarcoma">mesenchymal chondrosarcomas</a>
-<li>cortical remodelling, thickening and <a href="/articles/periosteal-reaction">periosteal reaction</a> are also useful in distinguishing between an enchondroma and low grade chondrosarcoma (see <a href="/articles/enchondroma-vs-low-grade-chondrosarcoma-2">enchondroma vs. low grade chondrosarcoma</a>)</li>
+<li>cortical remodelling, thickening and <a href="/articles/periosteal-reaction">periosteal reaction</a> are also useful in distinguishing between an enchondroma and low grade chondrosarcoma (see <a href="/articles/enchondroma-vs-low-grade-chondrosarcoma-2">enchondroma vs. low grade chondrosarcoma</a>)</li>
~~-<li>cortical breach, seen in 88% of longbone chondrosarcoma, c.f. only 8% of enchondromas</li>~~
+<li>cortical breach, seen in ~90% of long bone chondrosarcoma, c.f. only ~10% of enchondromas</li>
-</ul><h5>Nuclear medicine</h5><p>Typically chondrosarcomas demonstrate increased uptake on <a href="/articles/bone-scan">bone scan</a>, seen in over 80% of cases, and usually the uptake is quite intense. This is useful in helping to distinguish a low grade chondrosarcoma from an enchondroma as the later has increased uptake in only 21% of cases, and usually to a lesser degree. (see <a href="/articles/enchondroma-vs-low-grade-chondrosarcoma-2">enchondroma vs. low grade chondrosarcoma</a>)</p><h4>Treatment and prognosis</h4><p>Prognosis varies with both grade and location. In general:</p><ul>
+</ul><h5>Nuclear medicine</h5><p>Typically chondrosarcomas demonstrate increased uptake on <a href="/articles/bone-scan">bone scan</a>, seen in over 80% of cases, and usually the uptake is quite intense. This is useful in helping to distinguish a low grade chondrosarcoma from an enchondroma as the latter has increased uptake in ~20% of cases, and usually to a lesser degree (see: <a href="/articles/enchondroma-vs-low-grade-chondrosarcoma-2">enchondroma vs. low grade chondrosarcoma</a>).</p><h4>Treatment and prognosis</h4><p>Prognosis varies with both grade and location. In general:</p><ul>
~~-<li>grade 1: 90% 5 year survival</li>~~
~~-<li>grade 3: 29% 5 year survival</li>~~
+<li>grade 1: 90% 5-year survival</li>
+<li>grade 3: 29% 5-year survival</li>

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