Benign Cartilage Forming Tumors Flashcards

1
Q

What is the most common benign skeletal tumor?

A

osteochondroma

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2
Q

An osteochondroma is also called ____

A

exostosis

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3
Q

What age group is primarily affected by osteochondroma?

A

skeletally immature individuals

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4
Q

What are the 2 types of osteochondroma?

A
  • pedunculated
  • sessile
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5
Q

Most cases of osteochondroma are ____

A

solitary

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6
Q

Less than 6 osteochondroma lesions is termed ____

A

solitary osteochondroma

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7
Q

What is the name for the multiple form of osteochondroma?

A

Hereditary Multiple Exostosis (HME)

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8
Q

More than 6 osteochondroma lesions is termed ____

A

Hereditary Multiple Exostosis (HME)

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9
Q

Hereditary Multiple Exostosis is also called ____

A

osteochondromatosis

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10
Q

What is a major difference between the solitary and multiple forms of osteochondroma in terms of possible outcomes?

A
  • solitary: <1% chance of malignant transformation
  • multiple: up to 25% undergo malignant transformation
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11
Q

If an osteochondroma undergoes malignant degeneration, what will it transform into?

A
  • chondrosarcoma (most likely)
  • osteosarcoma
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12
Q

What is the etiology of osteochondromatosis?

A

autosomal dominant hereditary disease
(EXT1 & EXT2 genes)

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13
Q

What is the etiology of solitary osteochondroma?

A

spontaneous development, no genetic component

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14
Q

Describe the appearance of a pedunculated osteochondroma

A
  • bony stalk capped by cartilage
  • should not be thicker than 10mm
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15
Q

Describe the appearance of a sessile osteochondroma

A
  • don’t often have cartilaginous cap
  • cartilage is intermingled
  • “hill” on bone
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16
Q

At what point is a pedunculated osteochondroma considered to undergo malignant transformation?

A

when >2mm thickness
(use MRI to evaluate)

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17
Q

What is the preferential location of osteochondromas?

A
  • knee is common
  • pelvis, scapula, ribs
  • metaphysis near growth plate of endochondral bones only
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18
Q

Describe the histological layers of a pedunculated osteochondroma

A
  • bone
  • hyaline cartilage
  • perichondrium
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19
Q

How would a patient with sessile osteochondroma present clinically?

A
  • generally painless (non-tender), non-mobile, potentially palpable lumps
  • tendinosis/bursitis
  • neurovascular bundle impingement
  • malignant transformation (rare)
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20
Q

How would a patient with pedunculated osteochondroma present clinically?

A
  • generally painless (non-tender), non-mobile, potentially palpable lumps
  • pathological fracture
  • tendinosis/bursitis
  • neurovascular bundle impingement
  • malignant transformation (rare)
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21
Q

How would a patient with Hereditary Multiple Exostosis present clinically?

A
  • begin forming both sessile & pedunculated osteochondromas ~3 years old
  • bowing/shortening of affected bones
  • limited ROM
  • multiple bones w/ exostoses (6-100s)
  • family Hx

(in addition to SSx of solitary osteochondroma)

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22
Q

What is the treatment for solitary osteochondromas?

A

surgical removal, generally once skeletally mature (will only stop growing once pt stops growing)

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23
Q

What is the treatment for Hereditary Multiple Exostosis?

A
  • surgical removal if clinical problems
  • correction of bone deformities (osteotomy)
  • address biomechanical issues
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24
Q

Name the 2 types of benign cartilaginous tumors

A
  • enchondroma
  • juxtacortical chondroma
25
Q

What are enchondromas comprised of?

A

hyaline cartilage

26
Q

What are juxtacortical chondromas comprised of?

A

hyaline cartilage

27
Q

What population is primarily affected by benign cartilaginous tumors?

A

<20 years

28
Q

Benign cartilaginous tumors form in ____ bones

A

endochondral

29
Q

What is the preferential location of enchondromas?

A

medullary cavity

30
Q

What is the preferential location of juxtacortical chondromas?

A

surface (cortical) of bone

31
Q

What is the etiology of benign cartilaginous tumors?

A

mutation in genes related to chondrocyte development

32
Q

If an enchondroma undergoes malignant degeneration, what will it turn into?

A

chondrosarcoma
(<1% chance in solitary lesion)

33
Q

Multiple enchondromas are referred to as ____

A

Ollier Disease
(has possible hereditary component)

34
Q

What is the chance of malignant transformation in Ollier Disease?

A

25%

35
Q

Multiple enchondromas plus soft tissue hemangiomas are referred to as ____

A

Mafucci Syndrome
(rare)

36
Q

Ollier Disease plus soft tissue hemangiomas are referred to as ____

A

Mafucci Syndrome
(rare)

37
Q

What is the chance of malignant transformation in Mafucci Syndrome?

A

50%

38
Q

What are the multiple forms of enchondroma called?

A
  • Ollier disease
  • Mafucci syndrome
39
Q

What is a hemangioma?

A

benign vascular tumor

40
Q

What is the most common benign tumor of the hands and feet?

A

Enchondroma

41
Q

How would a patient with an enchondroma present clinically?

A
  • usually asymptomatic (incidental finding)
  • Patho Fx (painful)
  • No Fx + pain = possible malignancy
42
Q

How would you differentiate between an enchondroma and a chondrosarcoma?

A

use MRI with contrast (inject with gadolinium) to highlight vascular structures
- enchondroma shows up normal
- chondrosarcoma shows high contrast (^angiogenesis)

43
Q

What are the radiographic characteristics of an enchondroma?

A
  • geographic lytic lesion w/ or w/out sclerotic border
  • narrow/short ZoT
  • 50% have matrix calcification
  • intact & thinned cortex, endosteal scalloping
  • deformities
44
Q

What is endosteal scalloping?

A

curvilinear edges (scalloped) of inside surface of bone (endosteum)

45
Q

How would a patient with Ollier Disease present clinically?

A
  • severe skeletal deformity
  • shortened bones
  • patho Fx
46
Q

What type of matrix calcification is seen in Ollier disease?

A

stippled calcification (hyaline cartilage)

47
Q

What is the pattern of destruction in Ollier disease?

A

geographic lytic

48
Q

Small, round lumps of calcium that form in a person’s veins, which may show up as white spots radiographically are called ____

A

phleboliths

49
Q

What are the radiographic characteristics of Mafucci syndrome?

A
  • multiple geographic lytic lesions
  • phleboliths outside of pelvic basin
50
Q

Multiple phleboliths outside of the pelvic basin indicates ____

A

hemangiomas

51
Q

How would a patient with Mafucci syndrome present clinically?

A
  • shortened bones
  • dense, purple-coloured skin
  • bone deformities
  • patho Fx
52
Q

What is the treatment for benign cartilaginous tumors?

A
  • none, unless symptomatic
  • monitor
  • surgical removal of disruptive juxtacortical chondromas
  • curettage of problematic enchondromas
  • treatment of patho Fx
  • monitor & treat for malignant degeneration
53
Q

What is the preferential location of chondroblastomas?

A

epiphyses (articular ends) & apophyses of endochondral bones

54
Q

What age group is primarily affected by chondroblastomas?

A

5-25 years

55
Q

How would a patient with a chondroblastoma present clinically?

A

joint pain that is difficult to recreate with ortho tests
(bone pain, not mechanical)

56
Q

What are the radiographic characteristics of chondroblastomas?

A

geographic lytic lesion in epiphyseal region
(slow-growing, well-defined borders)

57
Q

What is the treatment for a chondroblastoma

A

surgical removal (curettage) due to severe pain

58
Q

A 35 year old patient with a history of a single sessile osteochondroma arising from the medial aspect of the distal femur presents with pain immediately adjacent to that site. Light touch is intact, muscle testing of the leg is 5/5, and pulses are strong. What is the most likely explanation for why there is pain?
A) neurovascular bundle impingement
B) fracture
C) malignant degeneration
D) tendinosis/bursitis

A

D