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Chondrosarcoma

Chondrosarcomas are malignant cartilaginous tumours that account for 20-27% of all primary malignant bone tumours

Epidemiology

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 phenomenon.

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 for rings and arcs calcification or popcorn calcification.  

Grading

Chondrosarcomas are divided into 3 (sometimes 4) grades based primarily on cellularity. (see Chondrosarcoma grading)

Sub types

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

Primary
Secondary

Arising from pre-existing cartilagenous lesions :

Distribution
  • long bones: 45% (the reason is because 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
  • 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 a the time of diagnosis, usually over 4 cm in diameter and over 10 cm in 50% of cases.

Plain film
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 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 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 calcificaiton, c.f. 60-78% on plain film.
  • endosteal scalloping
  • cortical breach, seen in 88% of longbone chondrosarcoma, c.f. only 8% 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 hyper intense c.f muscle
    • iso to slightly hypo intense c.f grey matter (see chondrosarcoma of the base of skull)
  • T2 - very high intensity in non mineralised / 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

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
  • location
    • long bones have a better prognosis than axial skeleton

Related articles

Bone tumours

The differential diagnosis for bone tumours is dependant on the age of the patient, with a very different set of differentials for the paediatric patient.

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