FIBROUS DYSPLASIA OSSIFYING FIBROMA

PATHOGENESIS  Postzygotic mutation GNAS-1 gene o True neoplasm resembling Focal

-early postzygotic phase = involve 3 tissues cemento-osseous dysplasia
-late postzygotic phase = skeletal system o Possible e/p:
 Continuous activation G-protein  cAMP production o PDL
increased o Mutationtumor suppressor gene HRPT-
 Affects endocrine function, osteoblast maturation and 2
melanocytic activity
CLINICAL FEATURES  Aged 3-15 y/o o 3rd -4th decade life peak
 Delayed eruption tooth o Female > Male
 Malposed tooth o Mand premolar-molar area
1. Monostotic o Facial asymmetry due to cortical
- Involves only one bone expansion
- If involves the craniofacial bones  Craniofacial type o Malposed tooth
2. Craniofacial
- Facial deformities
- Visual disturbances
3. Polystotic
- Involves more than one bone
- Shepherd Crook deformity
- Symptoms : i) Mazabraud – intramuscular myxoma
ii) Jaffe-Leichenstein – PFD +Cafe au lait
iii) MCAS – JL + Endocrine dysfunction
RADIOLOGICAL  Ground glass/ orange peel appearance o Well defined uni/multilocular
FEATURES  Unilocular/ multilocular radiolucency o Radilucent/radiopaque
o Buccolingual bony expansion
o Adjacent teeth root resorption
HISTOPATHOLOGICAL  Bony trabeculae arranged in Chinese pattern like  Cellular fibrous tissue stroma
FEATURES arrangement  Osteoid/Cementum spherules
 No peripheral osteoblastic rimming of trabeculae  Heterogenous mineralisation
 Fibrous connective tissue stroma
 Homogenous mineralisation
 PAGET’S DISEASE/OSTEITIS DEFORMANS OSTEOSARCOMA
PATHOGENESIS  Increased osteoclastic sensitivity to IL-6, RANK-L o Radiation
and 1,25-dihydroxyvitamin D o Alkylating agent
 Abnormal and excessive bone resorption o Paget’s disease
 Accelerated and abnormal bone deposition o P53,RB-1
 Other e/p: o Hormonal growth spurts in adolescents
-Viral; inclusion bodies similar to paramyxoviruses o Hereditary retinoblastoma
-Inflammation
-Autoimmune
-Genetics
CLINICAL FEATURES  Elderly >50y/o o Bimodal age distribution:
 Bone pain i)Adolescence
 Weight bearing bone affected; bowing deformity ii) > 60y/o
known as Simian stance o Male
 Skull o Site:
-Platybasia, descending cranium due to soft base I) Axial and flat bones
of skull ii) Jaw
-Leontiasis ostea o Classification: i) Central/intermedullary
 Deaf and visual impairment due to nerve ii) Juxtacortical
compression iii) Extraskeletal
 Frontal bossing o Pain
 Migration of teeth o Swelling
 Enlargement alveolar ridges o Tooth mobility
o Nasal obstruction (maxilla)

RADIOLOGICAL  Osteolytic phase o Radiopaque/Mixed

FEATURES - Decreased radiodensity;radiolucent areas o Ill-defined borders
- Coarse trabeculae o Sunburst appearance
 Osteoblastic phase o Codman’s triangle at periosteum of long bones
- Sclerotic area o Symmetrical widening PDL space
-Cotton wool appearance due to difference in
 mineralisation of bone
-Hypercementosis tooth
HISTOPATHOLOGICAL  Osteolytic phase  Osteoid production and fibrous CT
FEATURES ->>> multinucleated large osteoclasts in  Tumor cells forming cellular disorganised bone
Howship’s lacunae -uniform round spindle w bizarre nucleic and
- resorption bone cytoplasmic shape
-behind leading edge, osteoclast  osteoblast  Types:
 Mixed phase i) Osteoblastic
- haphazard new bone formation due to repeated ii)Chondroblastic
bone formation and resorption iii) Fibroblastic
 Osteoblastic phase  Low grade tumors
- bone formation disorganised woven pattern -min cellular atypia
- peripheral osteoblastic rimming present in bony -abundant bone formed
trabeculae
- Reversal lines seen: alt between resorptive
and formative bone
- Mosaic pattern/ jigsaw puzzle pattern