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Clear cell chondrosarcoma

Dr Jeremy Jones ◉ and A.Prof Frank Gaillard ◉ ◈ et al.

Clear cell chondrosarcomas are a subtype of chondrosarcoma constituting 1-2% of all chondrosarcomas. They are typically low-grade (see chondrosarcoma grading) and get their name from the presence of clear cell chondrocytes which contain abundant vacuolated cytoplasm due to the presence of glycogen.

On this page:

Article:

Epidemiology
Clinical presentation
Pathology
Radiographic features
Treatment and prognosis
Differential diagnosis
Related articles
References

Images:

Cases and figures
Imaging differential diagnosis

Epidemiology

They typically present in patients in their 3^rd to 5^th decade, with men being affected twice as often as women (M:F ~ 2:1 ).

Clinical presentation

Symptoms persist for an average duration of one year, suggesting slow growth of the lesion.

Pathology

In approximately half of the cases, conventional chondrosarcomatous elements are also present. Macroscopically, hemorrhage and cyst formation are common, and radiographically matrix calcification is uncommon.

Location

The lesion is most often in an epiphyseal location (in contradistinction to conventional chondrosarcoma, which is usually metaphyseal-diaphyseal).

tubular long bones: 85-90%
- proximal femur: 55-60% (commonest)
- proximal humerus: 10-15%
- distal femur or proximal tibia: 10-15%

Radiographic features

Plain radiograph

On radiographs, clear cell chondrosarcomas are predominantly lytic and expansile.

endosteal irregularity is not infrequent
periosteal reaction does not occur
soft tissue extension and cortical break are usually seen only if a pathologic fracture occurs
low reported incidence of calcified matrix
in up to 20% of cases, a peripheral rind of sclerosis is apparent (simulating a benign lesion)

MRI

Typically reported signal characteristics on MR imaging include:

T1: homogeneous intermediate signal intensity
T1 C+ (Gd): may show heterogeneous enhancement
T2: heterogeneous high signal intensity

Treatment and prognosis

A clear cell chondrosarcoma is a relatively slow-growing, low-grade malignant tumor. It carries a much better prognosis than a conventional chondrosarcoma. Because of the relatively benign appearance of the lesion on radiographs, surgical intervention is often delayed. Distant metastases have, however, been reported in the lung, brain, and bones.

Differential diagnosis

chondroblastoma: a particular feature of clear cell chondrosarcomas is a predilection for the epiphysis, in which case they mimic chondroblastomas; distinguishing between the two can be difficult (see Chondroblastoma vs. clear cell chondrosarcoma)

References

Bone tumours

The differential diagnosis for bone tumors is dependent on the age of the patient, with a very different set of differentials for the pediatric patient.

bone tumors
- bone-forming tumors
- cartilage-forming tumors
- fibrous bone lesions
- bone marrow tumors
- other bone tumors or tumor-like lesions
- skeletal metastases
  - morphology
  - location
  - impending fracture risk
    - Harrington criteria
    - Mirel classification
- staging
  - AJCC staging of musculoskeletal tumors
  - Enneking surgical staging system

Article information

rID: 6194

System: Musculoskeletal

Tag: bone tumour, cases, refs

Synonyms or Alternate Spellings:

Clear cell chondrosarcomas

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Cases and figures

Case 1Case 1

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Case 2: in proximal humerusCase 2: in proximal humerus

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