6. Fibro-Osseous Lesions of Bone Flashcards
What is the common histology of Fibro-Osseous Lesions of Bone?
Spindly cellular, fibrous stroma, containing variable amounts of some mineralized material (bone, cementum, or combo), not dystrophic calcification
What is the age of pts with Fibrous Dysplasia?
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
Where does Fibrous Dysplasia occur?
ANY BONE in the body
Posterior jaws favored if it occurs in the jaws
What is the pathogenesis of Fibrous Dysplasia?
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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
What form of Fibrous Dysplasia occurs when the G-protein mutation occurs postnatally (as an infant)?
Monostotic Fibrous Dysplasia
Only 1 bone is affected
What form of Fibrous Dysplasia occurs when the G-protein mutation occurs in early embryonic life?
Polyostotic Fibrous Dysplasia
The single mutated cell is destined to become multiple different bones
What form of Fibrous Dysplasia occurs when the G-protein mutation occurs super early in embryonic life?
Albright Syndrome
- The same G-protein also controls:
- Melanin production - Cafe-au-lait spots - Coast of Maine
- Endocrine secretions - precocious puberty in females
What is the appearence of Monostotic Fibrous Dysplasia?
Doesn’t cross the midline
What is the severe expression of Polyostotic FIbrous Dysplasia?
Albright Syndrome
- Fibrous Dysplasia of many bones
- Precocious puberty in female
- Cafe-au-lait spots - coat of maine (jagged)
What is Jaffe Lichenstein Syndrome?
Milder Albright Syndrome
No precocious puberty in females
What are the characteristics of Juvenile Aggressive Fibrous Dysplasia? (5)
- Young kids
- Rapid swelling
- Gross deformity = Leontiasis Ostea
- Also in Paget’s Disease
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Destruction of tooth buds, it will move teeth
- This doesn’t occur in regular FD
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Interference with swallowing and breathing
- Surgery must be done
What are the 3 ways Fibrous Dysplasia can appear radiographically?
- Ground Glass RO
- Multi-locular RL
- Mixed RL with Mottled RO
What are the radiological findings in Fibrous Dysplasia? (4)
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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
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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
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Multi-focal Growth
- Teeth are not displaced relative to each other, like in a neoplasm
What is the Histological presentation of Fibrous Dysplasia? (4)
- 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
What is the histologic features of Craniofacial Complex Fibrous Dysplasia?
- 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
What is the treatment for Fibrous Dysplasia?
-
Delayed surgical recontouring of deformity until after puberty, or it will regrow more aggressively
- Bone is easily shaved “carboard consistency”
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Orthodontics is easily accomplish
- Teeth move through fibrous dysplastic bone like butter
In Fibrous Dysplasia what can occur with multiple surgeries and radiation?
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Sarcomatous Transformation of fibrous dysplasia bone into a:
- Osteosarcoma
- Fibrosarcoma
What lesion is the rarest of the fibro-osseous group?
Ossifying Fibroma
What population is affected by Ossifying Fibroma?
Females 3rd - 4th decade
Where do Ossifying Fibromas occur?
ONLY IN JAWS
Prefers mandibular premolars + molars