Chondrosarcoma

[ Home ] [ Up ]

Pages Below:

• malignant
• tumour of cartilage
• primary
  • very uncommon
  • found in children
  • arises centrally in bone
• secondary
  • arises from benign cartilage defects
    • osteochondroma (surface)
    • enchondroma (intramedullary)
• grow slowly
• arise in
• long bones,
• pelvis,
• ribs
• spine
• may be well differentiated
• occur in 5th and 6th Decades
  • patient's with Ollier's Disease or Maffucci syndrome (multiple enchodromas + haemangiomas) at much higher risk and present in 3rd and 4th decade

Presentation

• High-grade

  • excruciating pain

• Low-grade

  • older patient complaining of hip pain and swelling

• Pelvic

  • urinary frequency/obstruction

  • groin muscle pulls

Investigations

• plain film

  • fusiform, lucent defect

  • scalloping of inner cortex

  • periosteal reaction

  • extension into soft tissue may be present 

  • punctate/stippled calcification of cartilage

• MRI

  • demonstrates intraosseous and soft tissue extension

  • evaluation of malignant change in osteochondromas 

    • cartilage cap normally <2cm

Pathology

• macrosopic

  • greyish white, lobulated mass

  • focal calcification

  • mucoid degeneration

  • necrosis

• microscopic

  • enlarged plump nuclei

  • multiple cells per lacunae

  • binucleated cell

  • hyperchomic nuclear pleomorphism

Grading

• 1 to 3

• higher grades have worse prognosis and early mets.

Types

• Mesenchymal chondrosarcoma

  • rare variant

  • bimiorphic histology

    • low grade cartilaginous cells

    • hypercellular, small, uniform, undifferentiated cells (resemble Ewings sarcoma)

  • predilection for spine, ribs and jaw

  • presents in 3rd decade

  • more common in females

  • metastasises to lungs, lymph node and other bone

• Clear cell chondrosarcoma 

  • is a malignant cartilage tumor that 

  • may be the adult variant of chondroblastoma.

  • rare, 

  • low-grade tumour

  • improved prognosis over other chondrosarcomas

  • found in the epiphysis of the femur and humerus

  • soft tissue invasion is rare. 

  • Clear cell chondrosarcoma has clear cells with vacuolated cytoplasm

  • cartilage matrix has significantly calcified trabeculae and giant cells

• Dedifferentiated chondrosarcoma 

  • most malignant form of chondrosarcoma

  • mix of low grade chondrosarcoma and high grade spindle cell sarcoma 

    • spindle cells are no longer identifiable as having a cartilage origin

    • dedifferentiated portion of the lesion may have histological features of malignant fibrous histiocytoma, osteosarcoma, or undifferentiated sarcoma

  • biphasic quality is evident on x-ray with 

    • areas of endosteal scalloping and cortical thickening 

    • areas of cortical destruction and soft tissue invasion.

  • 5 year survival of 10%

Treatment. 

• wide surgical excision. 

• very limited role for chemotherapy or radiation

• Biopsies must be planned with future tumor excision in mind. 

Prognosis

• Patients with adequately resected low grade chondrosarcomas have an excellent survival rate. 

• survival of patients with high grade tumours depends on the location, size and stage of the tumour.

[ Home ] [ Up ]