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54 Cards in this Set
- Front
- Back
What four anatomic locations are most commonly affected by chordoma?
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Hip
Knee Shoulder Hand |
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What is the typical location within bone?
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Metaphysis of long bones
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How does chordoma appear on plain radiographs?
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Stippled area within bone
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Is the bone scan positive or negative?
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Positive
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What is the histologic appearance?
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Benign cartilage
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What is the usual treatment?
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None usually necessary (if asymptomatic)
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Where are periosteal chondromas seen on bones?
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On the surface of long bones
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What is the plain radiographic appearance of periosteal chondroma?
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Cortical defect with adjacent spicules
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If treatment is required for symptomatic periosteal chondroma, what is it?
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Marginal excision
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What two syndromes are associated with multiple chondromas? What are their characteristics?
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Ollier syndrome (multiple chondromas, 30% malignancy rate)
Mafucci syndrome falso soft tissue angiomas, 100% malignancy rate) |
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What is the inheritance pattern for these syndromes?
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None; sporadic mutation
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What three anatomic locations are most commonly affected by osteochondroma?
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Hip
Knee Shoulder |
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What is key to note about the medullary cavity on radiographs? Cortex?
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Bony medullaiy space continuous with that of the exostosis
Cortex of the lesion is confluent with that of the bone |
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What is the general rule about size of cartilage cap? What is an exception?
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2 to 3 mm thick cartilage cap
May be thicker in growing children |
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Osteochondroma is histologically similar in appearance to what?
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Normal physis
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How frequently does malignant degeneration occur?
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Fewer than 1% of patients
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What are two warning signs of malignant degeneration?
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Pain without mechanical symptoms
Growth after puberty with large cartilage cap |
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lf malignant degeneration does occur, what is the usual grade of the malignancy?
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Low grade
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What syndrome is associated with multiple exostoses?
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Multiple hereditary exostoses (MHE)
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What is the inheritance?
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AD
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What is the mutation?
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EXT 1,2 genes
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What is the overall risk of malignancy with MHE?
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10%
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If a malignancy does develop, what is it and where is it most common?
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Secondary chondrosarcoma
Especially around shoulder |
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What are the two key clinical features of the characteristic wrist deformities?
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Ulna short
Distal radius angular malalignment |
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What population is most commonly affected by chondroblastoma?
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Young adults
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What three anatomic locations are most commonly affected?
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Hip
Knee Shoulder |
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What is the typical location within bone?
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Epiphysis
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Quick review: what other tumor is commonly found at the same location within bone?
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Giant cell tumor
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What is the MRI appearance of chondroblastoma?Similar to what two nonneoplastic conditions?
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Lytic epiphyseal lesion with
surrounding edema Similar to osteochondritis dissecans (OCD) and osteomyelitis |
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What are the two key histologic features?
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Chondroblasts: polygonal cells
Giant cells scattered throughout |
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What are two key phrases on the histology description?
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Cobblestone
Chicken wire |
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What are the two components of the usual treatment of chondroblastoma?
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Curettage and graft
Consider treating with phenol also |
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How frequently does chondroblastoma metastasize?
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Metastasizes to lungs in 2% of patients
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Metastatic pattern and frequency are similar to what other lesion?
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Giant cell tumor
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How often does chondroblastoma recur?
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10%
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What gender is most commonly affected?
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Male
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What bone is most commonly affected?
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Tibia
May occur in other long bones as well |
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What is the typical clinical presentation of chondromyxoicl fibroma?
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Pain
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What are the classic plain radiographic findings?
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Eccentric lytic metaphyseal lesion
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What are the classic histologic features?
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Myxoid (white) with cartilage (blue);see lobules of cartilage/fibrous
tissue/ giant cells |
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What is the usual treatment of chondromyxoid flbroma?
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Marginal resection
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What are the two components of the plain radiographic appearance of chondrosarcoma?
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Bone destruction (lytic)
Mineralization (if chronic) |
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What grade are most clrondrosarcomas?
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Low
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How is chondrosarcoma treated?
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Wide resection
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How can a dedifferentiated chondrosarcoma be identified histologically?
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Biphasic appearance with low-and high-grade areas
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What are the two components of the treatment of dedifferentiated chondrosarcoma?
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Wide resection
Chemotherapy |
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Quick review: what is the cortical involvement in enchondroma versus chondrosarcoma?
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Enchondroma: partial cortical
involvement, but remains within cortex Chondrosarcoma: through cortex sometimes with a soft tissue mass |
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What are the x-ray changes over time in the adult in enchondroma versus chondrosarcoma?
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Enchondroma: no radiographic progression with time
Chondrosarcoma: progresses with time |
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ls there pain in enchondroma and chondrosarcoma?
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Enchondroma usually painless
Chondrosarcoma often painful (especially at night) |
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Clear cell chondrosarcoma occurs at what location within bone?
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Epiphyseal
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Can chordomas be found outside of the sacrum?
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Yes; especially in vertebral bodies
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What characteristic cells are seen on histology?
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Physaliferous cells (large clear cells)
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What are the two components of the treatment of chordoma?
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Resection
Radiation |
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How often do metastases occur?
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50%
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