Chondrosarcoma

Subtypes

  • Classic Intramedullary
  • De-differentiated Chondrosarcoma
  • Clear Cell Chondrosarcoma
  • Mesenchymal Chondrosarcoma

Intramedullary Chondrosarcoma

Epidemiology

  • Affects older patients (>45 years)
  • Slightly more common in males

Aetiology

  • Unknown
  • Associated with:
    • Expression of telomerase and RT gene
    • Presence of metastases
  • Rarely arises from solitary enchondroma (1%)
  • Increased risk in:
    • Ollier’s disease (20%)
    • Maffucci’s disease (100%)

Presentation

  • Slow-growing painful mass
  • Bowel and bladder symptoms if pelvic involvement

Clinical Features

  • Prognosis depends on grade:
    • Most are low-grade
    • High-grade tumors, especially de-differentiated, are aggressive

Location

  • Common sites:
    • Shoulder
    • Pelvis
    • Proximal femur
    • Hand

Imaging Features

  • Features vary by grade but typically include:
    • Cortical erosion and breach
    • Cortical thickening
    • Chondroid spiculing and matrix
    • Soft tissue mass
    • Periosteal reaction
  • Bone Scan: High uptake in all grades and subtypes
  • MRI and CT: Critical for assessing soft tissue extent and surgical planning

Pathology

  • Diagnosis may be challenging; histology is often featureless.
  • Hallmark: Invasion of trabecular bone
  • Other features:
    • Large pleomorphic cells
    • Binuclear nuclei
    • Plump and large nuclei

Management

  • Not radiosensitive or chemosensitive
  • Low-grade tumors: Treated with intralesional curettage and grafting
  • High-grade tumors:
    • Wide or radical surgical resection
    • Amputation for pelvic lesions if necessary
    • Lung metastases resectable if feasible

Outcomes

  • Prognosis depends on grade:
    • Grade 1: 90%
    • Grade 2: 60%
    • Grade 3: 30%
    • De-differentiated: 10%
  • Low-grade tumors may progress slowly over 20+ years.

De-differentiated Chondrosarcoma

  • Same locations as intramedullary chondrosarcoma
  • Highly malignant
  • Comprises low-grade chondrosarcoma overlaid on a spindle cell tumor (e.g., osteosarcoma)
  • Features:
    • Bimorphic radiologic and histologic appearance
  • Treatment:
    • Wide or radical resection for chondroid component
    • Chemotherapy for sarcoma component

Clear Cell Chondrosarcoma

  • Rare
  • Radiologic Features:
    • Lytic, well-demarcated lesion in the epiphysis of long bones
    • Differential diagnosis: Chondroblastoma
  • Characteristics:
    • Low or intermediate grade
  • Treatment:
    • Same as other chondrosarcomas
  • Prognosis:
    • 80% long-term survival

Mesenchymal Chondrosarcoma

  • Very rare
  • Occurs in flat bones; lesions are lytic
  • Affects younger patients rather than older adults
  • Prognosis:
    • 50% survival at 5 years
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