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54 Cards in this Set

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