Chondrosarcoma
Synonyms: Conventional chondrosarcoma, primary chondrosarcoma
IDH mutation status important for prognosis and emerging therapy
Quick Facts
Behaviour
Malignant
Category
Bone
Grade
Variable
Synonyms
- Conventional chondrosarcoma
- primary chondrosarcoma
Category
Bone
Behaviour
Malignant
Grade
Variable
Gender
M = F
Tissue of Origin
Cartilage
Epidemiology
- Second most common primary Malignant Bone tumour in adults
- Peak incidence 4th-6th decades
- Predilection for pelvis, femur, humerus
- IDH wild-type generally grade 2-3; IDH-mutant grade 1
Clinical Features
- Pain
- Swelling
- Slow-growing
- Constitutional symptoms if advanced
Location
- Pelvis (most common)
- Distal femur
- Proximal humerus
- Ribs
- Proximal femur
- Tibia
Imaging
- Plain radiograph: mixed lytic and sclerotic lesion with Rings and arcs calcification
- CT chest: pulmonary staging
- MRI: assess soft tissue and marrow involvement
Pathology
- Malignant cartilaginous differentiation
- Atypical chondrocytes
- Grading 1-3
- IDH1/2 mutations present in many
Genetics
- IDH1/2 mutations in 60%
- CDKN2A deletion in High-grade
- TP53 mutations in some
Treatment
- Wide surgical excision: primary treatment
- Chemotherapy and radiotherapy limited role
- Targeted therapy with IDH inhibitors under investigation
Prognosis
- Grade-dependent: 90% 5-year for grade 1, 50% for grade 3
- IDH-mutant tumours may have better prognosis
Key Points
- IDH mutation status important for prognosis and emerging therapy
- Wide excision critical
- Chemotherapy generally not effective
Workup - Blood Tests
- FBC, U&E, LFTs
- LDH
Workup - Local Imaging
- Plain radiograph
- MRI with contras of whole bone to assess local extent
- CT may aid in characterisation of bony detail
Workup - Biopsy
- Core needle biopsy: adequate tissue for grading
- IDH mutation testing recommended
Workup - Staging
CT chest: pulmonary metastases
Workup - Other
MDT at Bone sarcoma centre
Follow-up Summary
- 1
Post-operative visit at 6 weeks
- 2
Year 1–2
3-monthly clinical examination, plain films of primary site, and CXR
- 3
Year 3–5
6-monthly clinical examination, plain films of primary site, and CXR
- 4
Year 6–10
Annual clinical examination, plain films of primary site, and CXR
- 5
Discharge at 10 years from surgery
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