DEFINITION AND PATHOGENESIS
- Primitive cells from physis compose the tumor
- Benign lesion occurring in characteristic locations, histologically confused with malignant lesions
- (Codman's tumor when in the proximal humerus)
- <1% of all bone tumors; 70% present in 2nd decade (22 ± 9.9 yrs average age reported)
- Pain, limitation of motion of the adjacent joint
- M:F = 2-3:1
- ~1/6 occur in each of these locations: proximal humerus, proximal tibia, distal femur, proximal femur
- Other sites
- scapula, patella, distal radius, distal humerus, proximal fibula
- Cauda equina syndrome in a lumbar location has been described
- Rare to have 2 separate or adjacent bones involved ± synchronously
- Occasionally will metastasize
- ESR may be ?
- Oncogenous osteomalacia has been reported
- 40% lucent eccentric epiphyseal(secondary ossification center) lesion only; periostitis (due to inflammatory reaction to chondroblastoma rather than mechanical stress across a weakened epiphysis) present in 47-60%
- Rare non-epiphyseal locations (metadiaphyseal) have been described
- Geographic borders, 70% with physis still open (rarely apophyseal in location)
- Unlike GCT, >½ have a sclerotic rim
- Calcific punctate densities best seen on CT when minute (can be seen on plain x-ray)
- Cortical margin intact, 20% with disruption of subchondral bone
- Lesion rarely can be extremely large or have a bubbly expanded appearance (esp. if recurrent)
- CT depicts matrix mineralization, marginal sclerosis, cortical erosion and any periosteal reaction soft tissue extension, joint effusion, and fluid levels
- MRI useful especially if extension beyond the physis, will reveal associated synovitis, surrounding edema, joint effusion, and occassionally cystic regions
- T2-weighted images: low to intermediate signal intensity
- Lobular internal architecture, fine lobular margins
- 77% with adjacent bone marrow edema, soft tissue edema
- Lobulated low signal intensity rim
- 92% with low signal intensity foci within tumor corresponding to calcifications
- 67% with adjacent joint effusion
- Bone scan is of limited value but shows moderate to intense uptake; rarely, multiple lesions have been reported
- Irregularly lobulated, ± hemorrhage, bluish gray, and gritty lesion that expands the epiphysis, ± degenerative cysts
- 20% of cases demonstrate joint seeding (via ligaments or previous surgery)
- Joint effusion, chronic synovitis, and tumor implants within the synovium
HISTOLOGIC AND MOLECULAR FEATURES
- Polygonal-shaped chondroblasts with distinct borders surrounded by dense eosinophilic chondroid matrix
- Lobulated and indented large nucleus
- Mitotic figures are rare and typical (1/3-5 hpf)
- Chondroid usually scant, rarely with ossification
- Calcification in a "chicken-wire" or honeycomb pattern not universally present
- Osteoclast-like giant cells almost universal, probably reactive
- Hemorrhage and necrosis, with foci of foam cells and cholesterol deposits, even ABC changes (15% reported)
- Hyaline cartilage sometimes seen
- Spindle-shaped stromal cells occasionally in foci
- Special stains
- Reticulin reveals a honeycomb pattern
- S100 usually +
- EM reveals microvillous processes, nuclear lobulation, large nucleoli, inner nuclear membrane nuclear substance deposits
- Ring chromosome 4 has been reported in one case
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- GCT (esp if larger lesion)
- Small cell osteosarcoma if chondroid mistaken for osteoid
- Infection (including TB)
- Eosinophilic granuloma
- Clear cell chondrosarcoma
- Mesenchymal chondrosarcoma
- Malignant lymphoma
- Degenerative cysts/osteonecrosis (esp in the femoral head with subchondral collapse)
- Fibrous dysplasia
- NOF (fibroxanthoma)
- Chondromyxoid fibroma
DISEASE COURSE AND TREATMENT
- Curettage, chemical tx with cryotherapy or phenolization, grafting or PMMA packing
- Recurrence reported about 5-35% (esp in difficult pelvic lesions)
- Local seeding can occur
- Penetration into the joint during curettage must be avoided
- Articular cartilage may be destroyed by the lesion
- Vascularized gluteus medius grafting after elevation of femoral head articular collapse has been reported
- Primary tx with percutaneous radiofrequency heat ablation has been reported
- Rarely metastasizes (reported up to 3%) to lungs as benign lesion
- Distant soft tissue metastases have been reported
- Malignant degeneration, "malignant chondroblastoma" of lesions have been reported as late as 18 yrs after resection
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