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

10. Fibrous Dysplasia and Pagets Flashcards

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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
may occur in areas of pathologic fx nonunion
Pseudoarthrosis
26
may look like FD in skull
Stage 2 of Paget’s
27
Paget’s thickens outer table of skull, whereas FD ____ outer table
thins
28
not commonly involved by FD
Spine
29
Paget’s Disease, Aka
osteitis deformans
30
Osteolysis followed by extensive attempts at repair
Paget’s Disease
31
Common in England, Australia, and New Zealand
Paget’s Disease
32
may have contributed to Beethoven’s | loss of hearing
Paget’s disease
33
Pain from microscopic fx, increased blood flow, joint destruction, mechanical destruction of bone, and Increase in hat size and shoe size
Paget's
34
Hallmark of Pagets
Bony enlargement
35
Shepherd’s Crook deformity, Sabre Shin deformity, and Basilar invagination
Pagets
36
May cause brainstem compression, syringomyelia, or | obstructive hydrocephalus
Basilar invagination
37
- Pseudofractures - Sabre Shin Deformity - Bony Enlargement - Out of focus appearance
Paget’s Disease
38
"cotton ball" or "cotton wool" appearance
Paget’s Disease
39
enlarged, sclerotic hemipelvis with joint space loss
Pagets
40
mc complication of Pagets
pathologic fracture
41
Local areas of demineralized bone in Pagets may result in
banana fracture (bilateral/symmetrical)
42
Spinal Stenosis from bone expansion in Paget’s Disease leads to
compressive neuropathy and Possible cauda equina
43
Weakness in legs, urinary incontinence, sensory disturbances
cauda equina
44
Due to hypercalcemia and hypercalciuria in Paget’s Disease
Urinary Calculus formation
45
Pagetic bone has 20X
normal vascularity
46
Extensive Paget’s may result in
cardiac congestive failure (Need at least 1/3rd of skeleton involved)
47
what % of patients with Paget’s disease are | asymptomatic?
90%
48
Paget’s Sarcoma prognosis
rapidly fatal
49
Paget’s begins in _______ region of long bone
subarticular
50
least likely bone to be affected by Paget’s
Fibula
51
Elevated Alkaline phosphatase and hydroxyproline
Paget's
52
- Bone destruction replaced by fibrous tissue and osteoid that may calcify – Possible hemorrhage and necrosis – Marked vascularity of bone
Paget’s Disease
53
“Mosaic Structure of Paget’s disease” results from
simultaneous Destruction and repair
54
Paget’s shows | increased uptake on bone scan due to
hypervascularity
55
Osteoclastic overactivity creates loss of bone | density and Osteoporosis Circumscripta
Paget’s Disease Stage 1
56
miexed and mc stage seen with cheap fibrous bone replacement following bone destruction
Paget’s Disease Stage 2
57
helps to distinguish Paget's disease from sclerotic metastases
osseous enlargement
58
Cotton Wool Appearance
Paget’s Disease Stage 2
59
picture frame vertebra and cotton-wool appearance (skull)
pagets stage 2
60
Abnormal lucency in the distal femur with a flame-shaped or “blade of grass” shaped proximal margin caused by the
`advancing lytic phase of Paget’s disease
61
Sclerotic or Ivory stage of paget's
Stage 3
62
Malignant Transformation seen in what stage of Paget's?
Stage 4 (Osteosarcoma mc)
63
Prostate metastasis elevates
acid phosphatase
64
Pagets affect on pelvis
- Cortical thickening - Expansion of pubis and ischium - Patchy sclerosis - Intermittent lucent defects - Increased trabeculae - rim or brim sign - Protrusio acetabuli
65
Begins as subarticular lesion, Shepherd’s Crook deformity, and Pseudofractures
Paget’s in the femure
66
blade of grass appearance aand sabre shin deformity
Paget's in the tibia
67
Paget's treatment
- Human and salmon calcitonin to inhibit bone resorption - Bisphosphonates - Supportive bracing to prevent deformities

Tri 6 - Tumors (12 decks)

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