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Wednesday, August 04, 2004
Giant cell tumours
Very rare in children below 10 years. Dominant in age of 20 and 45.
Radiological grading system
Type 1: 'quiescent', well-defined margin with sclerotic rim.
Type 2: 'active', well-defined margin w/o sclertotic rim, expansile.
Type 3: 'aggressive, ill-defined margin with cortical destruction.
* no relation with histologic finding.
> Matrix calcification occasionaly in long standing case.
> Giant cells are not neoplstic. Mono-nuclear cells are thought to be neoplastic.
> They produce RANKL, which stimulates formation and maturation of osteoclasts from osteoclast precursors.
> ABC formation occurs in 10% of cases.
> In one-third of cases, intravascular plugs are found (not prognostic significance)
Local reccure occurs in approximately 25% of patients.
Radiological grading system
Type 1: 'quiescent', well-defined margin with sclerotic rim.
Type 2: 'active', well-defined margin w/o sclertotic rim, expansile.
Type 3: 'aggressive, ill-defined margin with cortical destruction.
* no relation with histologic finding.
> Matrix calcification occasionaly in long standing case.
> Giant cells are not neoplstic. Mono-nuclear cells are thought to be neoplastic.
> They produce RANKL, which stimulates formation and maturation of osteoclasts from osteoclast precursors.
> ABC formation occurs in 10% of cases.
> In one-third of cases, intravascular plugs are found (not prognostic significance)
Local reccure occurs in approximately 25% of patients.
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Consider Chondroblastoma in patient with open growth plate. Chondroblastoma is a rare, benign tumor derived from chondroblasts. It is found in the epiphysis of long bones, usually of the lower extremity. The most common site is the distal femur followed by the proximal femur, proximal humerus and proximal tibia. The tumor has a preference for males over females and the mean age of presentation is approximately 20 years old. The tumor may have behavior not normally associated with benign tumors including pulmonary metastases as well as local invasion of bone and soft tissue.
Clinically, chondroblastoma presents as pain near a joint without history of trauma. A secondary synovitis can be induced by the tumor, but pathological fracture is extremely rare.
The diagnosis of chondroblastoma can usually be made by radiograph. when the age of the patient and location of the lesion are considered. The most common site for chondroblastoma is the epiphysis. The lesion is lytic with well defined margins and can be from 1-6cm in size. Scalloping or expansion of cortical bone may be present. Fine calcifications, either punctate or in rings, may be visible. Cysts are present about 20% of the time and both MRI and CT can define the fluid levels. CT is also useful for defining the relationship of the tumor to the joint, integrity of the cortex, and intralesional calcifications. The differential diagnosis includes enchondroma, central chondrosarcoma and aneurysmal bone cyst.
Reference
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Clinically, chondroblastoma presents as pain near a joint without history of trauma. A secondary synovitis can be induced by the tumor, but pathological fracture is extremely rare.
The diagnosis of chondroblastoma can usually be made by radiograph. when the age of the patient and location of the lesion are considered. The most common site for chondroblastoma is the epiphysis. The lesion is lytic with well defined margins and can be from 1-6cm in size. Scalloping or expansion of cortical bone may be present. Fine calcifications, either punctate or in rings, may be visible. Cysts are present about 20% of the time and both MRI and CT can define the fluid levels. CT is also useful for defining the relationship of the tumor to the joint, integrity of the cortex, and intralesional calcifications. The differential diagnosis includes enchondroma, central chondrosarcoma and aneurysmal bone cyst.
Reference
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