fibre-osseous lesions of the jaw Flashcards
1
Q
classification dysplastic
A
- Fibrous dysplasia
- Cemento-osseous dysplasia
- Hereditary
- Familial gigantiform cementoma
- Non-hereditary
- Focal cemento-osseous dysplasia
- Florrid cemento-osseous dysplasia
- Periapical cemento-osseous dysplasia
- Hereditary
2
Q
fibrous dysplasia
A
developmental and sporadic condition
3
Q
classification idiopathic
A
Paget’s disease of the bone
4
Q
classification neoplastic
A
cementoossifying fibroma
5
Q
pathogenesis of fibrous dysplasia
A
Postzygotic mutation (occur in postnatal life) – GNAS1 gene
Replacement of normal bone by excessive proliferation of cellular fibrous connective tissue intermixed with irregular bone trabeculae
6
Q
monostotic FD incidence
A
- 80-85% of all FD cases
- peak incidence: second decade of life
- no gender predilection
7
Q
monoatomic FD presentation
A
- single lesion on a single bone
- painless, slow growing swelling
- tooth displacement may occur
- truly monostotic lesion in the mandible
8
Q
craniofacial FD
A
unique to maxilla: single lesion involving multiple bones that are close in proximity
9
Q
radiographic features - FD
A
- ground glass appearance
- poorly demarcated: “blends into surrounding bone”
- early lesion: radioluscent/mottled
10
Q
radiographic features - FD - Mn
A
- expansion of lingual and buccal plates
- bulging of the inferior border
- superior displacement of the inferior alveolar canal
- narrowing of periodontal ligament space
- ill defined lamina dura
11
Q
radiographic features - FD - Mx
A
- superior displacement of the Mx sinus floor
- obliteration of the Mx sinus
- increase in bone density
12
Q
polyostotic FD pathogenesis
A
- GNAS1 gene mutation occurs in later stages of embryological development, effecting only skeletal progenitor cells
- when mutation occurs in undifferentiated stem cells early in embryonic life
=> melanocytes, osteoblasts and endocrine cells will carry the mutation
13
Q
polystotic FD with cafe au last spots
A
Jaffe-Lichenstein syndrome
14
Q
polystotic FD with cafe au last spots + endocrinopathies
A
Mcune-Albright syndrome
15
Q
endocranopathies
A
- sexual precocity (most common)
- pituitary adenoma
- hyperthyroidism
16
Q
mazabraud syndrome
A
FD and intramuscular myxomas associated with an increase risk of malignant transformation
17
Q
clinical features of polystotic FD
A
- affects two on more bones
- facial asymmetry
- pathological fractures
- deformity
- hypophosphatemia
=> renal phosphate wasting
=> renal influences of circulating FGF23 produced by affected bone - cafe au lait spots:
irregular
well defined
unilateral macules
DDX: cafe au last spots of neurofibromatosis (smooth, regular borders)
18
Q
polyostotic FD histopathological features
A
Irregular curvilinear thin trabeculae of woven & lamellar bone
Bone is metaplastic therefore no osteoblastic rimming
Cellular loosely arranged fibrous stroma
Calcified spherules/acellular calcified
deposits/psammomatoid bodies
Monotonous pattern of arrangement
Lesional bone fuses with normal bone at
the periphery
Jaw FD undergoes maturation unlike long bone FD resulting in both woven and lamella bone
19
Q
treatment and prognosis
A
- stabilisation post skeletal maturation
- contouring for aesthetic reason and lesions that continue to grow
- bisphosphonate therapy
20
Q
cement-osseous dysplasia pathogenesis
A
- tooth bearing areas of the jaw
- most common fibro-osseos lesion
- periodontal origin
- defect in bone remodelling
- hormonal
21
Q
clinical features of focal FD
A
- single site of involvement
- female predilection
- mean age: 38 years
peak incidence: 3rd-6th decade of life - predominant site: mn posterior
- asymptomatic
- <1.5cm
- (e)edentulous areas
22
Q
clinical features - periodical OD
A
- periapical region of the anterior Mn
- solitary/multiple
- female predilections
- 70% cases - Africans
- associated teeth are vital
- asymptomatic
- circumscribed radioluscent periapical tooth areas
- radioluscent => radioluscent opaque => radiopaque with a radioluscent rim
- self limiting
- <1cm
- no cortisol expansion
23
Q
histopathological features of OD
A
- fragments of cellular – mesenchymal/ connective tie composed of fibroblasts and collagen fibres
- numerous blood vessels
- tabeculae or woven and lamellar bone
- cementum-like particles
- heterogeneity from lesion to lesion and field to field of the same lesion
- with maturation bone trabeculae thicken and resemble ginger roots
lobular masses of acellular and disorganised cement-osseous material
24
Q
clinical features - florid OD
A
- multifocal involvement
- posterior mx and mn
often concurrent involvement of an mn - predominantly African women
- middle aged to older adults
- bilateral
- ussually symmetrical involvement
- most commonly all 4 quadrants involved
- asymptomatic/ symptomatic
- dull pain
- minimal cortisol expansion
25
radiograghical features of focal OD
- circumscribes radioluscent => radioluscent/radiopaque => radiopaque with a radioluscent rim
- instant periodontal ligament space
- (e)edentulous areas
- may be associated with simple bone cysts
26
diagnosis of OD
clinic-radiological correlation very important
27
macroscopic features of osseous dysplasia
- fragmented gritty tissue
- firmly attached to adjacent bone
28
macroscopic features of ossifying fibroma
- one or several large masses
- clean separation from adjacent bone: "shells out"
29
histopathological features of osseous dysplasia
- bulky trabeculae of bone
- no osteoblastic rimming
- cementum like particles are irregular, no brush border
- retraction from adjacent stroma
- haemorrhage throughout lesion
30
ossifying fibroma
- delicate trabeculae of bone
- osteoblastic rimming
- cementum like particles are ovoid, regular, have a brush border
tabeculae of bone in close association with stroma
- haemorrhage along lesions margin
31
treatment prognosis - osseous dysplasia
- non-neoplastic
- no treatment required
- regular recall examination
- prophylactic enforcement of good oral hygiene
32
ossesous dysplasia - symptomatic
-secondary inflammatory/infective component
- osteomyelitis involving dysplastic, hypocellular, hypovascular, hypoxic bone
33
avoid biopsy and extractions
onset of symptoms associated with exposure to oral cavity
34
familial gigantiform cementoma
Autosomal dominant
Few sporadic cases
No gender predilection
Whites
Early onset(1st decade of life)
Rapid and expansive growth
Limited to jaws
Multifocal involvement of both jaws
Facial deformity
Impaction
Malposition
Malocclusion
35
familial gigantifrom cementoma may be associated with
- adenomas of the uterus
- anaemia
- elevated serum alkaline phosphatase
- bone fragility (pathological fractures
36
radiographic features - familial gigantiform cementoma
- multiple radiolucencies in the periapical regions
- radioluscent => radioluscent/radiopaque with radioluscent rim
- inovolvement of all four quadrants
- cortisol expansion
37
familial gigantiform cementoma - histopathological features
same features as osseous dysplasia
38
familial gigantiform cementoma - treatment and prognosis
extensive resection and reconstruction
39
ossifying fibroma (cement-ossifying fibroma) - non-neoplastic
peripheral ossifying fibroma
40
ossifying fibroma (cement-ossifying fibroma) - neoplastic
- conventional ossifying fibroma
- juvenile ossifying fibroma
- juvenile trabeculae ossifying fibroma
- juvenile psammomatoid ossifying fibroma
41
conventional ossifying fibroma - clinal features
Mn>Mx
Most common site : premolar-molar area
Painless swelling
Facial asymetry
Pain and paraesthesia rarely
42
conventional ossifying fibroma radiographic features
Well defined
Unilocular/multilocular
Radiolucent→ radiolucent- radiopaque →radiopaque
Root divergence
Root resorption
43
conventional ossifying fibroma - histopathological features
Well demarcated
May be encapsulated
Cellular fibrous tissue
Mineralised material
Trabeculae of osteoid, lamellar and woven bone
Basophilic, poorly cellular spherules with a brush border
Variation in hard tissue
44
conventional ossifying fibroma - treatment and prognosis
Enucleation
Surgical resection and bone grafting
Good prognosis
45
juvenile trabeculae ossifying fibroma
Mineralised tissue predominantly trabeculae of bone
46
juvenila psammomatoid ossifying fibroma
predominantly psammomatoid deposits
psammomatoid more common than trabeculae
47
clinical and radiological features of juvenile ossifying fibroma
Rapid growth
Well circumscribed
Radiolucent → radiolucent- radiopaque
Ground glass appearance
Mean age for trabecular JOF: 11 years
Mean age for psammomatoid JOF: 22 years
Slight male predilection
Mn
48
juvenile ossifying fibroma - histopathological features
Cellular fibrous connective tissue
Haemorrhage
Multinucleated giant cells
Juvenile Trabecular Ossifying Fibroma
Cellular osteoid
Plump osteocytes and osteoblasts
Osteoblastic rimming
Juvenile Psammomatoid Ossifying Fibroma
Concentric spherical ossicles of varying shape and size
49
juvenile ossifying fibroma - treatment and prognosis
- complete local excision
- wider resection
50
Paget's disease of bone - clinical features
Abnormal deposition and
resorption of bone
Cause is unknown
?inflammatory, genetic,
hormonal
Rare in Africa
Older adults
Male predilection
Asymptomatic
Chronic
Slowly progressive
Monostotic< polyostotic
Symptomatic: bone pain
Joint pain and limited mobility
Thickened, weakened, and
enlarged bone
Increase in the circumference of
the head
Nasal obstruction
Enlarged turbinates
Obliterated sinuses
Enlarged alveolar ridges – “dentures don’t fit any longer”
Multiple diastema
Hypercementosis
Neurological complications due to bone encroachment on cranial nerves
Visual disturbances Deafness
Bone fractures
Malignant transformation→ osteosarcoma
51
Paget's disease of the bone - radiographic features
Early stages:
Large areas of radiolucency(osteoporosis circumscriptions)
Osteoblastic Phase:
Cotton wool appearance
due to patchy bone
sclerosis
Hypercementosis
52
Paget's disease of the bone - histopathological features
Alternating bone deposition and resorption
Osteoclasts
Osteoblasts
Vascular fibrous CT
Reversal lines
Jigsaw/ mosaic appearance of bone
53
Paget's disease of the bone - diagnosis
Elevated levels of serum alkaline phosphatase
associated with increased osteoblastic activity
Normal blood calcium and phosphorus
Histopathological examination unnecessary
54
Paget's disease of the bone - treatment and prognosis
Asymptomatic: no treatment
Symptomatic
Bone pain
Calcitonin/Bisphosphonates
