Bone Diseases II Flashcards
What are the most common benign cemento-osseus lesions?
Fibrous dysplasia
Cemento-osseous dysplasias Sub-types: Periapical cemental
Focal cemento-osseous
Florid cemento-osseous
Cemento-ossifying fibroma Sub-types: Conventional Juvenile Active
What is fibrous dysplasia?
A benign non-neoplastic, tumour-like, developmental defect in bone formation
It is caused by a fibrous proliferation forming disorderly malformed woven bone which is structurally weak.
Where is fibrous dysplasia most commonly found?
Ribs
Femur
Tibia
Pelvis
Craniofacial
What age is fibrous dysplasia most commonly seen?
Onset typically seen in childhood - adolescence
What are the clinical features of fibrous dysplasia?
Single or multiple bony lesions
Slow growing
Painless
Often quiesces at puberty
Genetics: GNAS I gene chromosome 20q13.1-2
What are the types of fibrous dysplasia?
Monostotic (70%) craniofacial (25%)
Polyostotic (25%) craniofacial (50%)
McCune-Albright Syndrome (3%) Endocrine abnormalities
What are the symptoms of fibrous dysplasia?
Painless swelling
Facial asymmetry
Malocclusion – displaced teeth
Headache
Hearing loss
Clinical labs: elevated alkaline phosphatase
What are the radiographic symptoms of fibrous dysplasia
Early lesions are radiolucent/mottled
GROUND GLASS APPEARANCE
Diffuse and poorly dilineated
Cortical expansion
Narrow PDL
Obscure lamina dura
What does fibrous dysplasia look like histologically?
Woven bone
Irregular trabeculae “chinese characters”
Fibrous stroma
No osteoblastic lining
Blends into normal bone - no capsule
Maturation into lamellar bone
What complications can arise from fibrous dysplasia?
Debilitating deformities
Aesthetic/psychosocial concerns
Malocclusion
Pathologic fractures
Cranial nerve and orbital involvement
Malignant degeneration
What causes malignant degneeration of areas with fibrous dysplasia?
- rare (< 1%)
- osteosarcoma
- avoid radiation therapy
How is fibrous dysplasia managed?
May not require treatment; conservative management is best.
Stabilizes with skeletal maturity
Diffuse involvement precludes excision
Cosmetic and functional deformity
Surgical recontouring/decompression
Regrowth (25-50% - younger patients)
What causes fibrous dysplasia?
This is caused by mutation in GNAS1 gene G protein increasing cAMP production in cells.
If it occurs early in development this results in a more pluripotent stem cell getting ti and thus a systemic condition results (McCune Albright syndrome) and if it occurs later in development it can become monostotic. If at an intermediate stage polyostotic.
What is periapical cemento-osseus dysplasia?
A benign non-neoplastic dysplastic process
Who typically gets periapical cemento-osseus dysplasia?
Incidence: relatively common
Age: middle age (30 – 50 years)
Gender: female > male (14:1)
Race: black
Where does periapical cemento-osseus dysplasia typically occur?
Mandible anteriorly in the periapical area.
What are the clinical symptoms of periapical cemento-osseus dysplasia?
Asymptomatic, vital teeth, non-expansible
What are the common radiographic findings of periapical cemento-osseus dysplasia?
Multiple, circumscribed apical radiolucencies. < 1cm
Variable in radiopacity based on maturation
Non-expansile
Teeth are vital.
What are the histological features of periapical cementoosseus dysplasia?
Spindled fibrous
stroma
Cementum calcified
matrix
Osseous calcified
matrix
Maturation
No inflammation
How is periapical cemento-osseus dysplasia managed?
Clinical – radiographic diagnosis
Benign self limiting process
No progressive growth
No treatment required
Prognosis: excellent
Avoid unnecessary endodontic therapy,
surgery or extractions
Who commonly gets focal cemento-osseus dysplasia?
4-5th decade
Most commonly in females
Caucasians
What causes focal cemento-osseus dysplasia?
Non-neoplastic - disordered growth of cementum and bone
What are the clinical features of focal cemento-osseus dysplasia?
Most common site: Posterior mandible
Asymptomatic
Solitary
Edentulous areas
Size: < 1.5 cm
What are the radiographic features of focal cemento-osseus dysplasia?
Mixed radiolucent - opaque well defined borders
What are the histological features of focal cemento-osseus dysplasia?
Benign fibro-osseus lesion
Cellular spindled fibrosis
Collagen fibers
Globular cementum matrix material
Woven osseous matrix material
Vascular channels
How is focal cemento-osseus dysplasia treated?
Curettage (Biopsy)
Watching and following up periodically.
What is the prognosis of focal cemento-osseus dysplasia like?
Excellent prognosis (limited growth potential)
What is florid cemento-osseus dysplasia?
Benign non-neoplastic dysplastic process