dr james c wittig, orthopedic oncologist, new york, new jersey
About Dr. Wittig
Clear Cell Chondrosarcoma
Clear Cell Chondrosarcoma

General Information

  • Malignant tumor
  • Comprised of neoplastic chondrocytes
  • With abundany, clear cytosplasm
  • Little interveining matrix
  • Foci of conventional chondrosarcoma may be present

Clinical Presentation

Signs/Symptoms:

  • Pain and swelling
  • There may be interference with motion
  • Often of long duration
Prevalence:
  • ~2% of all chondrosarcomas
  • 2 to 1 male predilection
Age:
  • Adolescence to old age
  • Most common in third and fourth decades
Sites:
  • Epiphyses of long bones
  • Most commonly proximal femur, proximal humerus, distal femur, proximal tibia

Radiographic Presentation

  • Usually osteolytic, expansile lesion
  • May be focally calcified
  • Often a sharp interface between tumor and surrounding bone
  • Sclerotic rim is uncommon
  • Overlying cortex is usually thin, but intact
Roll over the images for more information
Geographic, epiphyseal lesion, skeletally mature, no sclerotic rim AP pelvis
T1-weighted MRI proximal femur: clear cell chondrosarcoma MRI T2 Weighted Image
bone scan: increased uptake in region of proximal femur, clear cell chondrosarcoma Left proximal femur: clear cell chondrosarcoma
Plain X-ray of clear cell chondrosarcoma of proximal femur Plain X-ray:   Proximal femur clear cell chondrosarcoma
Proximal humerus clear cell chondrosarcoma

Gross Pathology

  • Cut surface
  • Typically lacks appearance of conventional chondrosarcoma
  • Usually red, soft and granular
  • Possibly multiple cysts
  • Small areas of gray or white cartilage may be irregularly interspersed

Microscopic Pathology

  • May have inconspicuous lobular pattern
  • Sometimes microlobules are separated by delicate fibrovascular stroma
  • Dominant cell is chondrocyte
  • Variably clear cytoplasm
  • Condensation of powdery cytoplasm near membrane or nucleus
  • Sharply defined cell borders
  • Nuclei are enlarged and rarely binuclear
  • Matrix formation is sparse
  • Small deposits of chondroid material between clear cells
  • May be focally calcified
  • More abundant m,atrix may be found in areas of conventional chondrosarcoma
  • These areas found in about half of clear cell variants


Roll over the images for more information
gross specimen histology: clear cell chondrosarcoma
pathology pathology
pathology pathology

Biological Behavior

  • Slightly aggressive
  • Destructive cortical penetration is rare, but possible
  • Recurrence rare
  • Metastasis somewhat common

Treatment and Prognosis

  • En bloc resection with wide margin of normal bone and soft tissue
  • Procedure of choice
  • Recurrence rare
  • Simple excision or curettage
  • 80% risk of local recurrence
  • Amputation always a possible back up
  • Metastasis
  • No microscopic or clinical differences from original lesion
  • Somewhat common
  • Even if treated with amputation
  • Death rate low to moderate
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