Fibrous Dysplasia & Paget's Disease

74 terms by skdaw9 

Create a new folder

Advertisement Upgrade to remove ads

Tumors

Fibrous Dysplasia and Paget's Disease are considered the "Great __" of bone disease.

"Great Imitators"

What are the 3 types of Fibrous Dysplasia?

1) Monostotic FB (70% ~14yo)
2) Polyostotic FB (27% ~11yo)
3) Polyostotic FB w/Endocrine abnormalities aka McCune-Albright Syndrome (3% ~8yo)

Briefly describe Fibrous Dysplasia?

Slow benign progression of bone being replaced by a weaker fibrous type of bone w/poorly formed bony trabeculae

In FD the bone scan is __ due to constant __.

"hot"
constant bone turnover

T/F FD is often asymptomatic and may present w/pathologic fracture.

True

What signs are seen w/FD?

1) Shepherd's Crook Deformity
2) Leontiasis Ossea
3) Leg length Discrepancy
4) Expansion of Rib
5) Cafe au Lait Spots

Define Shepherd's Crook Deformity.

Coxa vara (smaller angle) bowing of the proximal femur

Define Leontiasis Ossea.

Enlargement and deformity of the cranial and facial bones

Bowing deformities and pathologic fractures are m/c seen in what type of FD?

Polyostotic FD

Define Cafe au Lait Spots seen with FD

-pigmented skin macules due to increased melanin/proliferation of melanocytes in the epidermis
-irregular margins = "Coast of Maine" appearance

FD has a ground-glass/smokey-foggy matrix that is due to what?

Lack of normal Cortical and Trabecular organization
Note: this causes Endosteal Scaloping

T/F Endosteal Scaloping is seen in both FD and Paget's

False
-only FD

FD starts in the __ or __ and spreads into the __, while Paget's starts __ and spreads up or down.

-Metaphysis or Diaphysis
-other
-Subcondrally

The mc etiology of benign rib lesions is?

Monostotic FD

T/F there is no transistion from Monostotic to Polyostotic FD type.

True

T/F there is a higher incidence of Pathologic fractures and deformities (Shepherd's Crook) in Monostotic FD

False
-in Polyostotic FD

Breifly define McCune-Albright Syndrome.

-Polyostotic FD with skin pigmentation and early premature sexual development
-Females only
-Prematue irregular menstrual bleeding at 5-6yo

Cherubism is a condition seen in FD patients. Breifly explain.

-Multilocular Cystic lesions with asymmetric distribution in the mandible and maxilla
-Affects Jaws/swelling of lower face and cheeks
-Soap Bubbly expansile cortical thinning with trabeculation
-Regresses during puberty

FD in the skull and maxilla is more __ due to higher concentration of bone spicules

Radiopaque

T/F FD can malignant degenerate to Fibrosarcoma?

True

Monostotic FD is m/c in __ and __.

Ribs, Proximal Femur

Monostotic FD starts where in the bone and spares what on the bone.

-Diametaphyseal
-Spares Subarticular Suface

T/F Radiolucency, Loculated trabeculation, ground glass/smokey appearance (thus no trabecular patterns), geographic cystic lesions are seen in Monostotic FD

True

T/F A thick sclerotic margin aka rind of sclerosis is seen in Paget's, in which Myeloma's or Metastasis does not have.

False
-seen in Monostotic FD

T/F Endosteal scalloping widening medullary canal, Bone Expansion, NO periosteal reaction unless pathologic fraction are seen Monostotic FD

True

If only slight or no uptake is seen on bone scan of FD this means you can what?

Rule out FD

Polyostotic FD is m/c in __, __, __, and __.

Pelvis, Extremities, Ribs, and Skull

Polyostotic FD __ the out table of the skull, where Paget's __.

Thins
Thickens

T/F the spine is commonly involved in FD

False

T/F In Polyostotic FD symptoms are usually from bowing deformities and pathologic fractures.

True

T/F Shepherd's Crook deformity, a ground glass appearance, endosteal scalloping, loculation of lesions, and pseudofractures along the covex surface of deformed cortex are roentgen findings in Monostotic FD.

False
-in Polyostotic FD

Polyostotic FD rib lesions cause what sign in the lungs?

Extrapleural sign

T/F Monostotic FD normally invovles the entire shaft of the bone.

False
-Polyostotic FD

Breifly describe Paget's Disease (aka Osteitis Deformans).

-Osteolysis followed by extensive repeated attempts at repair
-Mostly(90%) Asymptomatic = incidental finding
-Fibula least likely affected

Age range for Paget's disease?

50+

In Paget's pain is from?

1) Microscopic Fx
2) Increased blood flow
3) Joint destruction
4) Mechanical destruction of bone

What is thee Hallmark sign in Paget's?

Bony Enlargement
-Increase in HAT and SHOE size normally seen
-Cortical Thickening
-Medullary Canal stenosis

What 3 deformities are seen in Paget's?

1) Shepherd's Crook deformity
2) Sabre Shin deformity
3) Basilar Invagination
4) Spinal Stenosis

What is Sabre Shin Deformity?

-anterior bowing of the shin
-Fibula not affected
-seen in Paget's

What is Basilar Invagination?

When C2 vertebra migrates in to the foramen magnum causing stenosis
-Brainstem Compression, syringomyelia, obstructive hydrocephalus
-Cranial N. palsies
-Deafness
-Bone incduced Basilar A. Syndrome w(HA, giddiness, vertigo, spinal cord ischemia)

What is seen w/Spinal Stenosis in Paget's?

-Bone expansion leads to compressive neuropathy
-possible Cauda Equina Syndrome
-Distorted soft bones or pathologicFx can = neuropathy
-Leg weakness, Urinary incontinence, sensory problems
-Protrusio Acetabuli

What are some Roentgen sign almost always seen in Paget's?

-Bony enlargement
-Cortical thickening
-Trabecular pattern more promininant (thickening)
-PathologicalFx or pseudoFx
-COTTON BALL/WOOL appearance
-Out of focus non-homogeneous PATCHY sclerotic appearance

T/F in Paget's a lot of times there is a fused appearance of bone, (ie SI, Femur Head)

True

T/F mc complication in Paget's is pathologic Fx.

True
-Banana like Fx affecting tubular bones usually weight bearing bones
-Ususally occur during stage 2 or 3

What are PseudoFx?

-Local areas of fibrous tissue replacing demineralized bone
-can result in complete Banana Fx
-Bilateral & Symmetrical lucent bands perpendicular to cortex

What is a Banana Fracture?

Transverse insufficiency fracture through the proximal femoral shaft

T/F Protrusio Acetabuli and leontiasis Ossea are common Paget's

True

T/F bone scans are "hot" with Paget's

True
-Due to hypervascularity
-Want to do plain films to confirm

In Paget's the Hip and Knee are commonly affected due to what?

Necrosis that caused articular cartilage resorption and replaced by Pagetic bone = arthrosis

Describe Malignant Degeneration of Paget's and what stage it occurs in?

-mc in femur, then humerus
-mc see Osteosarcoma (usu lytic)
-Stage 4

T/F in Pagets Cannon-Ball/Pulmonary Metastasis to the lungs is common = early death.

True

T/F Metastatic disease typically doesnt affect Pagetic bone.

True

Paget's usually has __ periosteal reaction, with soft tissue mass and pathologicFx being __.

-has NO periosteal reaction
-common

Paget's has a definite __ in alkaline phosphatase which can be a ddx to FD, which is less seen in.

Elevation
-20x normal

Pathology of Paget's?

-Bone destruction replaced by fibrous tissue and osteiod that may calcify
-Possible hemorrhage and necrosis
-Hypervascularity of bone
-Destruction and repair occur repeatedly, reulting in the "Mosaic Structure of Paget's"

4 Stages of Paget's Disease?

1) Osteolytic/Destructive
2) Destruction and Repair (M/C)
3) Rare (can occur outside of other stages in itself)
4) Malignant Transformation

Pagets of the Skull is know as__ and is seen in what stage?

-Osteoporosis Circumscripta
-Stage 1

Describe Stage 2 Paget's

-MC stage
-Destruction and Production (Repair) of bone
-Cheap fibrous bone replacing destroyed bone
-Cortical thickening
-Increased radiopacity and enhanced trabecular patterns w/interspersed radiolucent areas
-Bone Enlargement

Cotton Ball/Wool appearance is seen in which stage of Paget's?

Stage 2

Seen in Paget's, what is a Picture Frame Vertebra?

Enlargement/cortical thickening of vertebra with sclerosis more prominent at the endplates

Describe "Blade of Grass" seen in Paget's

Abnormal lucency in th distal femur w/a flame-shaped or "Blade of Grass" shaped proximal margin
-Due to advancing lytic phase of Paget's

What is seen in Stage 3 Paget's?

-Sclerotic or IVORY stage
-usu seen in innominate or vertebrae
-may or may not have bony enlargement

What are all the radiographic findings in Paget's Disease?

1) change in bone density
2) coarsened trabeculae
3) cortical thickening
4) bone enlargement
5) subarticular location
6) pathologic fx
7) pseudo fx
8) bowing deformities
9) sharp lytic area w/short zone of transition
--osteoporosis circumscripta in skull

T/F Paget's always begins at one end of a bone in the subarticular region, and progresses towards other end.

True

Paget's in the skull you see?

-map like resorption of bone
-inner table is not involved, but outter table of skull is resorbed from within (thicker)
-sutures are destroyed

What are the stages of Paget's in the skull?

1) Osteoporosis Circumscripta
2) Cotton Wool appearance = fuzzy, poorly defined margin of sclerotic areas
--irregular patches of bone in thickened diploe
--M/C stage

Paget's in the spine you see?

-Usu see stage 2 but stage 3(ivory vertebra) also seen
-Enlarged vertebra w/Rim of thickened cortex = Picture Frame appearance
-Accentuated Vertical Trabeculae

Paget's in the pelvis you see?

-commonly affected and usually seen in stage 2
-cortical thickening
-pubis and ischium expansion
-patchy sclerosis
-intermittent lucent defects
-increased trabeculae
-Rim/Brim Sign
-Protrusio Acetabuli

What is the Rim/Brim sign seen in the pelvis of Paget's?

Cortical thickening of the pelvic brim w/obliteration of Kohler's teardrop

Paget's in the femur you see?

-usu seen in stage 2
-begins as subarticular lesion and progresses toward other end
-ddx: FD begins in diametaphysis
-expanded corticies
-coarse trabecular patterns
-pseudo fx along covex side of subtrochanteric region of the femoral neck
-Shepherd's Crook deformity (varus deformity of hip)

Paget's in the tibia you see?

-Radiolucent defect beginning in subarticular end of proximal tibia extending down the shaft in a "candle flame/V-shaped" appearance("Blade of Grass")
--indicates advancing osteoclastic resorption
-Anterior bowing of tibia (Sabre Shin Deformity)
--extensive Paget's

What is used to ddx Paget's from Blastic/sclerotic metastasis?

-Bone Enlargement
-Squaring off of anterior vertebral body margins
-Endplate Thickening
-Occurs in subarticular end of bone

DDx osteolytic or osteoblastic metastatic carcinoma from Paget's.

-Lack of cortical thickening
-Frequent cortical disruption
-Lack of bone expansion (unless 1° thyroid or kidney carcinomoa)

DDx Hemangioma of bone from Paget's

-Vertical striations of hemangioma may resemble coarse vertical trabeculae of Paget's
-No endplate thickening

Please allow access to your computer’s microphone to use Voice Recording.

Having trouble? Click here for help.

We can’t access your microphone!

Click the icon above to update your browser permissions above and try again

Example:

Reload the page to try again!

Reload

Press Cmd-0 to reset your zoom

Press Ctrl-0 to reset your zoom

It looks like your browser might be zoomed in or out. Your browser needs to be zoomed to a normal size to record audio.

Please upgrade Flash or install Chrome
to use Voice Recording.

For more help, see our troubleshooting page.

Your microphone is muted

For help fixing this issue, see this FAQ.

Star this term

You can study starred terms together

NEW! Voice Recording

Create Set