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Tri 6 - Tumors > 10. Fibrous Dysplasia and Pagets > Flashcards

10. Fibrous Dysplasia and Pagets Flashcards

1
Q

Three types of Fibrous Dysplasia

A
  1. Monostotic fibrous dysplasia 70% ~age 14
  2. Polyostotic fibrous dysplasia 27% ~ age 11
  3. Polyostotic fibrous dysplasia with endocrine abnormalities
    (McCune-Albright Syndrome) ~3% ~ age 8
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2
Q

Slowly progressing benign disorder of fibro-osseous tissue throughout life replacing normal bone tissue

A

Fibrous Dysplasia

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3
Q

“Great Imitators of Bone Disease”

A

Fibrous Dysplasia and Paget’s disease

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4
Q

Polyostotic FD may have

A

bowing deformities and pathologic fx

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5
Q

Shepherd’s Crook deformity and Leg length discrepancy

A

Polyostotic FD

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6
Q

Enlargement and deformity of cranial

and facial bones

A

Leontiasis Ossea

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7
Q

Dark brown pigmented skin macules due to increased melanin in epidermis

A

Café au Lait Spots (Coast of Maine appearance)

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8
Q

ground-glass matrix

A

Fibrous Dysplasia

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9
Q

ground-glass matrix is
due to lack of
normal

A

cortical and

trabecular organization

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10
Q

most common etiology of benign rib lesions

A

Fibrous dysplasia

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11
Q

Main Pathologic Process of Café au lait spots is proliferation of

A

Melanocytes

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12
Q

Higher incidence of pathologic fx and Shepherd’s Crook deformity

A

Polyostotic FD

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13
Q

polyostotic FD with skin

pigmentation and precocious sexual development in Females

A

McCune-Albright Syndrome

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14
Q

occasional increase in alkaline

phosphatase and osteocalcin

A

Fibrous dysplasia

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15
Q

Multilocular cystic lesions with asymmetric distribution in mandible and maxilla and lower face swelling

A

cherubism

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16
Q

“Eyes raised to heaven attitude”

A

cherubism

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17
Q

Familial multilocular cystic disease of the jaws

A

cherubism

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18
Q

Bone is replaced by abnormal proliferation of fibrous tissue and poorly formed bony trabeculae of woven bone

A

Fibrous Dysplasia

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19
Q

Monostotic FD is mc in

A

ribs and proximal femur

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20
Q

Radiolucent Loculated, trabeculated, Ground glass or smokey appearance and Spares subarticular surface of bone

A

Monostotic FD

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21
Q

Thick sclerotic margin in Monostotic FD, aka a

A

rind of sclerosis

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22
Q

Symptoms of Polyostotic FD are usually from

A

bowing deformities and pathologic fx

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23
Q

Incomplete septa or ridges in Polyostotic FD result in

A

loculation of lesions

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24
Q

along convex surface of deformed cortex

A

Pseudofractures

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25
Q

may occur in areas of pathologic fx nonunion

A

Pseudoarthrosis

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26
Q

may look like FD in skull

A

Stage 2 of Paget’s

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27
Q

Paget’s thickens outer table of skull, whereas FD ____ outer table

A

thins

28
Q

not commonly involved by FD

A

Spine

29
Q

Paget’s Disease, Aka

A

osteitis deformans

30
Q

Osteolysis followed by extensive attempts at repair

A

Paget’s Disease

31
Q

Common in England, Australia, and New Zealand

A

Paget’s Disease

32
Q

may have contributed to Beethoven’s

loss of hearing

A

Paget’s disease

33
Q

Pain from microscopic fx, increased blood flow, joint destruction, mechanical destruction of bone, and Increase in hat size and shoe size

A

Paget’s

34
Q

Hallmark of Pagets

A

Bony enlargement

35
Q

Shepherd’s Crook deformity, Sabre Shin deformity, and Basilar invagination

A

Pagets

36
Q

May cause brainstem compression, syringomyelia, or

obstructive hydrocephalus

A

Basilar invagination

37
Q
  • Pseudofractures
  • Sabre Shin Deformity
  • Bony Enlargement
  • Out of focus appearance
A

Paget’s Disease

38
Q

“cotton ball” or “cotton wool” appearance

A

Paget’s Disease

39
Q

enlarged, sclerotic hemipelvis with joint space loss

A

Pagets

40
Q

mc complication of Pagets

A

pathologic fracture

41
Q

Local areas of demineralized bone in Pagets may result in

A

banana fracture (bilateral/symmetrical)

42
Q

Spinal Stenosis from bone expansion in Paget’s Disease leads to

A

compressive neuropathy and Possible cauda equina

43
Q

Weakness in legs, urinary incontinence, sensory disturbances

A

cauda equina

44
Q

Due to hypercalcemia and hypercalciuria in Paget’s Disease

A

Urinary Calculus formation

45
Q

Pagetic bone has 20X

A

normal vascularity

46
Q

Extensive Paget’s may result in

A

cardiac congestive failure (Need at least 1/3rd of skeleton involved)

47
Q

what % of patients with Paget’s disease are

asymptomatic?

A

90%

48
Q

Paget’s Sarcoma prognosis

A

rapidly fatal

49
Q

Paget’s begins in _______ region of long bone

A

subarticular

50
Q

least likely bone to be affected by Paget’s

A

Fibula

51
Q

Elevated Alkaline phosphatase and hydroxyproline

A

Paget’s

52
Q
  • Bone destruction replaced by fibrous tissue and osteoid
    that may calcify
    – Possible hemorrhage and necrosis
    – Marked vascularity of bone
A

Paget’s Disease

53
Q

“Mosaic Structure of Paget’s disease” results from

A

simultaneous Destruction and repair

54
Q

Paget’s shows

increased uptake on bone scan due to

A

hypervascularity

55
Q

Osteoclastic overactivity creates loss of bone

density and Osteoporosis Circumscripta

A

Paget’s Disease Stage 1

56
Q

miexed and mc stage seen with cheap fibrous bone replacement following bone destruction

A

Paget’s Disease Stage 2

57
Q

helps to distinguish Paget’s disease from sclerotic metastases

A

osseous enlargement

58
Q

Cotton Wool Appearance

A

Paget’s Disease Stage 2

59
Q

picture frame vertebra and cotton-wool appearance (skull)

A

pagets stage 2

60
Q

Abnormal lucency in the distal femur with a flame-shaped or
“blade of grass” shaped
proximal margin caused by the

A

`advancing lytic phase of Paget’s disease

61
Q

Sclerotic or Ivory stage of paget’s

A

Stage 3

62
Q

Malignant Transformation seen in what stage of Paget’s?

A

Stage 4 (Osteosarcoma mc)

63
Q

Prostate metastasis elevates

A

acid phosphatase

64
Q

Pagets affect on pelvis

A
  • Cortical thickening
  • Expansion of pubis and ischium
  • Patchy sclerosis
  • Intermittent lucent defects
  • Increased trabeculae
  • rim or brim sign
  • Protrusio acetabuli
65
Q

Begins as subarticular lesion, Shepherd’s Crook deformity, and Pseudofractures

A

Paget’s in the femure

66
Q

blade of grass appearance aand sabre shin deformity

A

Paget’s in the tibia

67
Q

Paget’s treatment

A
  • Human and salmon calcitonin to inhibit bone resorption
  • Bisphosphonates
  • Supportive bracing to prevent deformities

Tri 6 - Tumors (12 decks)

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