6. Fibro-Osseous Lesions of Bone Flashcards

1
Q

What is the common histology of Fibro-Osseous Lesions of Bone?

A

Spindly cellular, fibrous stroma, containing variable amounts of some mineralized material (bone, cementum, or combo), not dystrophic calcification

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2
Q

What is the age of pts with Fibrous Dysplasia?

A

1st – 2nd decade

  • Once it forms it isn’t going away, can see it in older people, it just developed a while ago
  • Grows with pt, peaking in puberty, it won’t regress after puberty, it stays the same size for life
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3
Q

Where does Fibrous Dysplasia occur?

A

ANY BONE in the body

Posterior jaws favored if it occurs in the jaws

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4
Q

What is the pathogenesis of Fibrous Dysplasia?

A
  • Developmental condition = somatic mutation in G protein (GNAS1):
    • G proteins act as an on/off switch, responsible for bone production, here it remains on
  • When it should stop bone production it doesn’t, so it keeps on producing bone
  • But it doesn’t mineralize bone, so the bone being produced continuously is soft, osteoid-like bone, that does not mature but keeps on growing
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5
Q

What form of Fibrous Dysplasia occurs when the G-protein mutation occurs postnatally (as an infant)?

A

Monostotic Fibrous Dysplasia

Only 1 bone is affected

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6
Q

What form of Fibrous Dysplasia occurs when the G-protein mutation occurs in early embryonic life?

A

Polyostotic Fibrous Dysplasia

The single mutated cell is destined to become multiple different bones

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7
Q

What form of Fibrous Dysplasia occurs when the G-protein mutation occurs super early in embryonic life?

A

Albright Syndrome

  • The same G-protein also controls:
    • Melanin production - Cafe-au-lait spots - Coast of Maine
    • Endocrine secretions - precocious puberty in females
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8
Q

What is the appearence of Monostotic Fibrous Dysplasia?

A

Doesn’t cross the midline

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9
Q

What is the severe expression of Polyostotic FIbrous Dysplasia?

A

Albright Syndrome

  • Fibrous Dysplasia of many bones
  • Precocious puberty in female
  • Cafe-au-lait spots - coat of maine (jagged)
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10
Q

What is Jaffe Lichenstein Syndrome?

A

Milder Albright Syndrome

No precocious puberty in females

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11
Q

What are the characteristics of Juvenile Aggressive Fibrous Dysplasia? (5)

A
  • Young kids
  • Rapid swelling
  • Gross deformity = Leontiasis Ostea
    • ​Also in Paget’s Disease
  • Destruction of tooth buds, it will move teeth
    • This doesn’t occur in regular FD
  • Interference with swallowing and breathing
    • Surgery must be done
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12
Q

What are the 3 ways Fibrous Dysplasia can appear radiographically?

A
  1. Ground Glass RO
  2. Multi-locular RL
  3. Mixed RL with Mottled RO
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13
Q

What are the radiological findings in Fibrous Dysplasia? (4)

A
  • Fusiform, tapered expansion
    • It can expand enough to cause facial deformity
    • Ground glass expansion of orbit and obliteration of the sinus
  • Diffuse, ill-defined, blending margins
  • Involves and incorporates cortical bone and lamina dura
    • Obliterates cortex, you can’t see the outline of the mandible, it has been incorporated - appears porous
    • Lose lamina dura
  • Multi-focal Growth
    • Teeth are not displaced relative to each other, like in a neoplasm
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14
Q

What is the Histological presentation of Fibrous Dysplasia? (4)

A
  • Immature woven bone forming directly from stroma, and forming irregular trabecular patterns = Chinese Characters
  • Don’t see osteoblasts producing it
  • Maturation Arrest - bone never matures, stays soft
  • Lamina dura replaced by dysplastic bone
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15
Q

What is the histologic features of Craniofacial Complex Fibrous Dysplasia?

A
  • It is a polyostotic fibrous dysplasia that acts differently
  • There is so much remodeling in the facial area that eventually the bone matures:
    • Start to get lamellaer bone and osteoblastic rimming
    • Lose the ability to discriminate it from Ossifying Fibroma or Osseous Dysplasia via biopsy
      • ​​if its next to a tooth you can differentiate FD - lamina dura is incorporated/replaced with dysplastic bone
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16
Q

What is the treatment for Fibrous Dysplasia?

A
  • Delayed surgical recontouring of deformity until after puberty, or it will regrow more aggressively
    • Bone is easily shaved “carboard consistency”
  • Orthodontics is easily accomplish
    • Teeth move through fibrous dysplastic bone like butter
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17
Q

In Fibrous Dysplasia what can occur with multiple surgeries and radiation?

A
  • Sarcomatous Transformation of fibrous dysplasia bone into a:
    • Osteosarcoma
    • Fibrosarcoma
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18
Q

What lesion is the rarest of the fibro-osseous group?

A

Ossifying Fibroma

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19
Q

What population is affected by Ossifying Fibroma?

A

Females 3rd - 4th decade

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20
Q

Where do Ossifying Fibromas occur?

A

ONLY IN JAWS

Prefers mandibular premolars + molars

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21
Q

What is the pathogenesis of Ossifying Fibroma?

A

True benign neoplasm of medullary bone

22
Q

What is the clinical presentation of an Ossifying Fibroma?

A

Single, painless, oval, bony swelling

23
Q

What is the form of Ossifying Fibroma that occurs in kids?

A

Juvenile Active Form of Ossifying Fibroma

24
Q

What are the characteristics of the Junvenile Active form of Ossifying Fibroma? (4)

A
  • Slowly expansile mass in children
  • Mostly in the maxilla
    • ​Normally Ossifying Fibroma is in the mandible
  • RL Cannonball
  • Shells out and rarely recurs
25
Q

What are the characteristics of Ossifying Fibroma that are radiographically distinguishable from Fibrous Dysplasia? (4)

A
  • Begins as a well-demarcated, ovoid RL
  • Increasing RO with age
  • Diverges roots
  • Thins and bulges cortex (dissolves cortex by pressure)
    • Not incorporating the cortex like in Fibrous Dysplasia
26
Q

What is the radiographic differential diagnosis for Ossifying Fibroma when it is RL? (3)

A
  • OKC
  • Periapical Cyst
  • Residual Cyst
27
Q

What is the radiographic differential diagnosis for Ossifying Fibroma when it is mixed RL/RO, encroaching cortex, not incorporating it?

A

Odontogenic Tumors + Cysts with RO

28
Q

What is the histology of Ossifying Fibroma? (4)

A
  • Fibrous capsule, that’s how you remove it in a smaller one
  • Very cellular, well demarcated border
  • Begins to acquire cementum-like calcified droplets (rounded, with concentric reversal lines)
  • Will produces bone, cementum & combinations of both, become indistinguishable from other Fibro-Osseous Lesions
29
Q

What is the treatment and prognosis for Ossifying Fibroma?

A

Shells out easily along its capsule

Rarely recurs

30
Q

What is the most common Fibro-Osseous Lesion?

A

Cemento-Osseous Dysplasia

31
Q

What is the pathogenesis of Cemento-Osseous Dysplasia?

A

Reactive process, but not sure what to

32
Q

What is the histology for all 3 subtypes of COD? (2)

A
  • Droplets of cementum & spicules of bone develop in cellular fibrous stroma
  • Eventually enlarging & coalescing as the lesion matures into a ginger-root pattern
33
Q

What population does Periapical COD (Cementoma) occur in?

A

80% in black females > 20 yrs

NEVER in White Males

34
Q

What is the location of a Periapical COD?

A

Must start as periapical to mandibular anterior’s

  • Can be individual or coalescing around several apices
  • Have to have teeth & be around apices
35
Q

What is the Clinical Presentation of Periapical COD? (4)

A
  • Once formed, will not resorb even w/ extraction
  • Asymptomatic
  • Assoc teeth are usually vital & seldom have restorations
    • Teeth can be non-vital but you can’t make the dx
  • Don’t have to be individual, can coalesce
36
Q

What is the radiographic appearance of the Periapical Cementoma?

A
  • Early RL Stage, when there is no RO
  • Stage 2 Cementoma, when RO start to form, where you get ground glass appearance or multiple chunks of RO
  • Stage 3 Cementoma, predominantly RO, with maybe a thin rim of RL
37
Q

What population is affected by Florid COD?

A

80% in black females > 20 yrs

38
Q

Where is Florid Osseous Dysplasia found?

A
  • Multifocal - periapical to multiple mandibular teeth
  • Above the mandibular canal
    • So does TBC
39
Q

What disease is associated with Traumatic Bone Cyst, and is the only time it can be seen in Adults?

A

Florid Osseous Dysplasia

40
Q

What is the clinical presentation of Florid COD? (2)

A
  • Lesion remains even when the associated tooth is extracted
  • Symptomatic
    • There is so much cemental material produced that it can cause swelling + sequestration
    • Only COD that is symptomatic
41
Q

What complications can occur with Florid OD?

A
  • Sclerotic cementum is pathologic & can’t resorb
  • If teeth are extracted or pt needs denture, the cemental masses become exposed & infected causing dull ache & Osteomyelitis
42
Q

What is the radiographic appearance of Florid COD? (3)

A
  • Lesions most visible around mandibular molar roots
  • Multiple areas of periapical RL with or without jagged RO
  • Induces Hypercementosis
43
Q

What is the treatment for Florid OD? (3)

A

Asymptomatic - don’t treat

  • Inform pt to take good care of their teeth, b/c if you need extractions & dentures the condition that you have now that is asymptomatic can become symptomatic
  • Dentures can cause Jaw Enlargement or Osteomyelitis
  • Osteomyelitis from dentures treated with antibiotics + debridement
44
Q

What population does Focal COD affect?

A

Black + white females > 20 yrs

45
Q

Where is Focal COD found?

A
  • Forms in edentulous mandibular extraction sites
  • Only one that is NOT periapical
  • Individual
46
Q

How does Focal COD differ from the other Cemento-Osseous Dysplasia? (4)

A
  • Only one seen regularly in white females
  • NOT Periapical
  • Always a Single Lesion
  • Edentulous site following extraction
47
Q

What are the similarities of Focal COD to the other Cemento-Osseous Dysplasias? (4)

A
  • Seen almost exclusively in middle aged females
  • Most cases in mandible
  • X-ray shows RL, mixed, or RO
  • Identical histology = gingger root pattern
48
Q

How is Focal COD different from Ossifying Fibroma? (3)

A

Focal COD…

  • Remains small < 1.5cm
  • Doesn’t encroach on cortex
  • At surgery it is gritty & vascular & doesn’t separate easily
    • Whereas OF shells out like a marble
49
Q

What are the similarities between Focal COD and Ossifying Fibroma? (5)

A
  • Well-demarcated RL, mixed lesion or RO
  • Mandible
  • Not assoc with teeth
  • Females
  • Identical Histology
50
Q

What is the treatment for Focal COD?

A

Difficult surgical removal

  • Gritty & vascular
  • Doesn’t shell out easily like ossifying fibroma