Chondrosarcoma
Updates to Article Attributes
Chondrosarcomas are malignant cartilaginous tumours that account for ~25% of all primary malignant bone tumours. They are most commonly found in older patients within the long bones and can arise de novo or secondary from an existing benign cartilaginous neoplasm. 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.
Epidemiology
The typical presentation is in the 4th and 5th 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 subtype (see below). In general, these tumours are multilobulated (due to hyaline cartilage nodules) with central high water content and peripheral endochondral ossification. This accounts not only for the high T2 MRI signal (see below) but also for rings and arcs calcification or popcorn calcification.
Grading
Chondrosarcomas are divided into three (sometimes four) grades based primarily on cellularity (see: chondrosarcoma grading).
Subtypes
Chondrosarcomas are either primary, arising de novo, or secondary and arise from a pre-existent cartilaginous mass (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
myxoid chondrosarcoma: usually intermediate grade
mesenchymal chondrosarcoma: usually high grade
Secondary
Arising from pre-existing cartilaginous lesions:
-
solitary osteochondroma
-
solitary enchondroma
Distribution
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long bones: 45% (the reason is that the cartilage is more abundant in the long, tubular bones)
pelvis: 25% especially around the triradiate cartilage
-
ribs: 8%
patients often younger than at other sites
anterior ribs/costochondral junction
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spine: 7%
greater male predominance 2-4:1
thoracic most common
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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 chondrosarcoma of the skull base)
hands and feet: rare cf. enchondromas
Radiographic features
Imaging findings vary somewhat with different subtypes 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 >4 cm in diameter and >10 cm in 50% of cases.
Plain radiograph
lytic (50%)
intralesional calcifications: ~70% (rings and arcs calcification or popcorn calcification)
endosteal scalloping: affecting more than two-thirds of the cortical thickness (cf. less than 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 (see enchondroma 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 calcification, cf. 60-78% on plain film
cortical breach, seen in ~90% of long bone chondrosarcoma, cf. only ~10% of enchondromas
soft tissue mass: tumour cellularity, and therefore density, increases with increased grade of the tumour
heterogenous contrast enhancement
MRI
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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 non-mineralised/calcified portions - the cartilage is a hydrophilic tissue with high water content6
gradient echo/SWI: blooming of mineralised/calcified portions
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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 - rings and arcs enhancement pattern6,7
most demonstrate heterogeneous moderate to intense contrast enhancement.
Nuclear medicine
Typically chondrosarcomas demonstrate increased uptake on bone scan, seen in over 80% of cases, and usually the uptake is quite intense. This is useful in helping to distinguish low-grade chondrosarcoma from an enchondroma as the latter has increased uptake in ~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 survival
grade 3: 29% 5-year survival
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location
long bones have a better prognosis than axial skeleton
-<li><p><a href="/articles/juxta-cortical-chondrosarcoma">juxtacortical chondrosarcoma</a>: low, intermediate or high grade</p></li>- +<li><p><a href="/articles/periosteal-chondrosarcoma-1">juxtacortical chondrosarcoma</a>: low, intermediate or high grade</p></li>
-<li><p><strong>T2:</strong> very high intensity in non-mineralised/calcified portions</p></li>- +<li><p><strong>T2:</strong> very high intensity in non-mineralised/calcified portions - the cartilage is a hydrophilic tissue with high water content<sup>6</sup></p></li>
- +<li><p>enhancement can be septal and peripheral rim-like corresponding to fibrovascular septation between lobules of hyaline cartilage - rings and arcs enhancement pattern<sup>6,7</sup></p></li>
-<li><p>enhancement can be septal and peripheral rim-like corresponding to fibrovascular septation between lobules of hyaline cartilage</p></li>
References changed:
- 6. Meyers S, Hirsch W, Curtin H, Barnes L, Sekhar L, Sen C. Chondrosarcomas of the Skull Base: MR Imaging Features. Radiology. 1992;184(1):103-8. <a href="https://doi.org/10.1148/radiology.184.1.1609064">doi:10.1148/radiology.184.1.1609064</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1609064">Pubmed</a>
- 5. Varma D, Ayala A, Carrasco C, Guo S, Kumar R, Edeiken J. Chondrosarcoma: MR Imaging with Pathologic Correlation. Radiographics. 1992;12(4):687-704. <a href="https://doi.org/10.1148/radiographics.12.4.1636034">doi:10.1148/radiographics.12.4.1636034</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1636034">Pubmed</a>
- 1. Murphey M, Walker E, Wilson A, Kransdorf M, Temple H, Gannon F. From the Archives of the AFIP: Imaging of Primary Chondrosarcoma: Radiologic-Pathologic Correlation. Radiographics. 2003;23(5):1245-78. <a href="https://doi.org/10.1148/rg.235035134">doi:10.1148/rg.235035134</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12975513">Pubmed</a>
- 7. Geirnaerdt M, Hogendoorn P, Bloem J, Taminiau A, van der Woude H. Cartilaginous Tumors: Fast Contrast-Enhanced MR Imaging. Radiology. 2000;214(2):539-46. <a href="https://doi.org/10.1148/radiology.214.2.r00fe12539">doi:10.1148/radiology.214.2.r00fe12539</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/10671608">Pubmed</a>
- 8. Engel H, Herget G, Füllgraf H et al. Chondrogenic Bone Tumors: The Importance of Imaging Characteristics. Rofo. 2021;193(3):262-75. <a href="https://doi.org/10.1055/a-1288-1209">doi:10.1055/a-1288-1209</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/33152784">Pubmed</a>
- 9. Aoki J, Sone S, Fujioka F et al. MR of Enchondroma and Chondrosarcoma: Rings and Arcs of Gd-DTPA Enhancement. J Comput Assist Tomogr. 1991;15(6):1011-6. <a href="https://doi.org/10.1097/00004728-199111000-00021">doi:10.1097/00004728-199111000-00021</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1939751">Pubmed</a>
- 1. Dähnert W. Radiology review manual. Lippincott Williams & Wilkins. (2003) ISBN:0781738954. <a href="http://books.google.com/books?vid=ISBN0781738954">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/0781738954?ie=UTF8&tag=radiopaediaor-20&linkCode=as2&camp=1789&creative=9325&creativeASIN=0781738954">Find it at Amazon</a><div class="ref_v2"></div>
- 2. Meyers SP, Hirsch WL, Curtin HD et-al. Chondrosarcomas of the skull base: MR imaging features. Radiology. 1992;184 (1): 103-8. <a href="http://radiology.rsna.org/content/184/1/103.abstract">Radiology (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/1609064">Pubmed citation</a><div class="ref_v2"></div>
- 3. Varma DG, Ayala AG, Carrasco CH et-al. Chondrosarcoma: MR imaging with pathologic correlation. Radiographics. 1992;12 (4): 687-704. <a href="http://radiographics.rsna.org/content/12/4/687.abstract">Radiographics (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/1636034">Pubmed citation</a><div class="ref_v2"></div>
- 4. Murphey MD, Walker EA, Wilson AJ et-al. From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23 (5): 1245-78. <a href="http://dx.doi.org/10.1148/rg.235035134">doi:10.1148/rg.235035134</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/12975513">Pubmed citation</a><div class="ref_v2"></div>
- 5. Geirnaerdt MJ, Hogendoorn PC, Bloem JL et-al. Cartilaginous tumors: fast contrast-enhanced MR imaging. Radiology. 2000;214 (2): 539-46. <a href="http://radiology.rsna.org/content/214/2/539.full">Radiology (full text)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/10671608">Pubmed citation</a><div class="ref_v2"></div>