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Bone Diseases II Flashcards

1
Q

What are the most common benign cemento-osseus lesions?

A

Fibrous dysplasia

Cemento-osseous dysplasias Sub-types: Periapical cemental
Focal cemento-osseous
Florid cemento-osseous

Cemento-ossifying fibroma
Sub-types: 
Conventional
Juvenile 
Active
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2
Q

What is fibrous dysplasia?

A

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.

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3
Q

Where is fibrous dysplasia most commonly found?

A

Ribs

Femur

Tibia

Pelvis

Craniofacial

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4
Q

What age is fibrous dysplasia most commonly seen?

A

Onset typically seen in childhood - adolescence

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5
Q

What are the clinical features of fibrous dysplasia?

A

Single or multiple bony lesions

Slow growing

Painless

Often quiesces at puberty

Genetics: GNAS I gene chromosome 20q13.1-2

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6
Q

What are the types of fibrous dysplasia?

A

Monostotic (70%) craniofacial (25%)

Polyostotic (25%) craniofacial (50%)

McCune-Albright Syndrome (3%) Endocrine abnormalities

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7
Q

What are the symptoms of fibrous dysplasia?

A

Painless swelling

Facial asymmetry

Malocclusion – displaced teeth

Headache

Hearing loss

Clinical labs: elevated alkaline phosphatase

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8
Q

What are the radiographic symptoms of fibrous dysplasia

A

Early lesions are radiolucent/mottled

GROUND GLASS APPEARANCE

Diffuse and poorly dilineated

Cortical expansion

Narrow PDL

Obscure lamina dura

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9
Q

What does fibrous dysplasia look like histologically?

A

Woven bone

Irregular trabeculae “chinese characters”

Fibrous stroma

No osteoblastic lining

Blends into normal bone - no capsule

Maturation into lamellar bone

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10
Q

What complications can arise from fibrous dysplasia?

A

Debilitating deformities

Aesthetic/psychosocial concerns

Malocclusion

Pathologic fractures

Cranial nerve and orbital involvement

Malignant degeneration

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11
Q

What causes malignant degneeration of areas with fibrous dysplasia?

A
  • rare (< 1%)
  • osteosarcoma
  • avoid radiation therapy
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12
Q

How is fibrous dysplasia managed?

A

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)

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13
Q

What causes fibrous dysplasia?

A

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.

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14
Q

What is periapical cemento-osseus dysplasia?

A

A benign non-neoplastic dysplastic process

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15
Q

Who typically gets periapical cemento-osseus dysplasia?

A

Incidence: relatively common

Age: middle age (30 – 50 years)

Gender: female > male (14:1)

Race: black

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16
Q

Where does periapical cemento-osseus dysplasia typically occur?

A

Mandible anteriorly in the periapical area.

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17
Q

What are the clinical symptoms of periapical cemento-osseus dysplasia?

A

Asymptomatic, vital teeth, non-expansible

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18
Q

What are the common radiographic findings of periapical cemento-osseus dysplasia?

A

Multiple, circumscribed apical radiolucencies. < 1cm

Variable in radiopacity based on maturation

Non-expansile

Teeth are vital.

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19
Q

What are the histological features of periapical cementoosseus dysplasia?

A

Spindled fibrous
stroma

Cementum calcified
matrix

Osseous calcified
matrix

Maturation

No inflammation

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20
Q

How is periapical cemento-osseus dysplasia managed?

A

Clinical – radiographic diagnosis

Benign self limiting process

No progressive growth

No treatment required

Prognosis: excellent

Avoid unnecessary endodontic therapy,
surgery or extractions

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21
Q

Who commonly gets focal cemento-osseus dysplasia?

A

4-5th decade

Most commonly in females

Caucasians

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22
Q

What causes focal cemento-osseus dysplasia?

A

Non-neoplastic - disordered growth of cementum and bone

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23
Q

What are the clinical features of focal cemento-osseus dysplasia?

A

Most common site: Posterior mandible

Asymptomatic

Solitary

Edentulous areas

Size: < 1.5 cm

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24
Q

What are the radiographic features of focal cemento-osseus dysplasia?

A

Mixed radiolucent - opaque well defined borders

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25
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
26
How is focal cemento-osseus dysplasia treated?
Curettage (Biopsy) Watching and following up periodically.
27
What is the prognosis of focal cemento-osseus dysplasia like?
Excellent prognosis (limited growth potential)
28
What is florid cemento-osseus dysplasia?
Benign non-neoplastic dysplastic process
29
What are the radiographic findings for florid cemento-osseus dysplasia?
Irregular lobular dense radiopacities Mixed radiolucent and radiopaque areas Diffuse involvement of maxilla and mandible Bilateral Symmetrical
30
What are the histolopathologic findings of florid cemento-osseus dysplasia?
Fibroblastic proliferation Dense sclerotic calcific masses Cementum matrix Woven bone Inflammatory infiltrate Simple bone cysts
31
How is florid cemento-osseus dysplasia managed?
Benign self-limiting process No treatment required Potential for significant complications Avoid exposure of calcific masses Maintenance of dentition Chronic osteomyelitis – pain, fistulae, dehiscence, sequestration Traumatic bone cysts
32
What is a cementoblastoma?
An odontogenic tumour that forms a mass of cementum or cementum like tissue and is continuous with the tooth root. Can be very destructive of tissue
33
Which teeth are often affected by cementoblastomas?
Usually a mandibular premolar or first molar
34
What are the clinical features of a cementoblastoma?
Tender, sometimes painful swelling Located on the buccal and lingual/palatal aspect of the alveolus
35
Who typically gets a cementoblastoma?
Under 30 years of age M:F 1.2:1
36
Which tooth is most commonly affected by a cementoblastoma?
Mandibular 1st molar 80% of them are in the mandible
37
What are the radiographic features of a cementoblastoma?
Radiopaque mass with a thin radiolucent rim attached to the roots of a tooth Tooth resorption, loss of root outline, and obliteration of the periodontal ligament space
38
What are the histological features of a cementoblastoma?
Sheets or trabeculae of cementum-like calcified tissue with variably prominent reversal lines, sometimes with a pagetoid appearance
39
How is cementoblastoma treated?
Surgery Recurrence 35 - 60%
40
How are odontogenic tumours classified?
Based on similarity between tumours and various stages of tooth development 1. Odontogenic epithelium without odontogenic ectomesenchyme 2. Odontogenic epithelium with odontogenic ectomesenchyme, with or without dental hard tissue formation 3. Odontogenic ectomesenchyme with or without included odontogenic epithelium
41
What benign tumours fall under the category of odontogenic epithelium without odontogenic ectomesenchyme?
Ameloblastoma Squamous odontogenic tumour Calcifying epithelial odontogenic tumour (Pindborg Tumour)
42
What benign tumours fall under the category of odontogenic epithelium without odontogenic ectomesenchyme +/- dental hard tissue?
Ameloblastic fibroma Ameloblastic fibro-dentinoma Ameloblastic fibro-odontome Adenomatoid odontogenic tumour Calcifying odontogenic cyst Complex odontome Compound odontome
43
What benign tumours fall under the category of odontogenic ectomesenchyme +/- odontogenic epithelium?
Odontogenic fibroma Myxoma [myxofibroma] Benign cementoblastoma ('true' cementoma)
44
Which odontogenic tumours are malignant?
Clear cell odontogenic carcinoma Malignant ameloblastoma Primary intra-osseus carcinoma Malignant variants of other odontogenic epithelial tumours Malignant changes in odontogenic cysts
45
What are the clinical features of ameloblastoma?
Slow growing, polycystic or monocystic
46
Who typically gets ameloblastoma?
More males after the age of 40
47
What is an osteoma?
A reactive, developmental (non-neoplastic) bone forming tumour
48
Where is an osteoma typically found?
Craniofacial bones: Skull and paranasal sinuses
49
What are the symptoms of an osteoma?
Often a solitary, exophytic mass of dense bone arising from periosteal or endosteal surface. Often asymptomatic and is a non-aggressive tumour with no malignant transformation
50
What is an osteosarcoma?
Malignant mesenchymal neoplasm Osteoid production by tumor cells Most common primary bone malignancy
51
What is the most common age of onset of an osteosarcoma? Is it male or female dominant?
2nd decade of life More common in males Male:Female 1.6:1
52
What are the most common sites for an osteosarcoma?
Metaphysis of long bones Distal femus, proximal tibia, humerus
53
What could potentially cause osteosarcoma?
Could be primary (most common primary bone malignancy) Secondary to: Paget’s disease of bone Fibrous dysplasia Irradiation Retinoblastoma – tumor suppressor gene (13q14) Osteochondromatosis Chronic osteomyelitis
54
Where on the head and neck is osteosarcome most commonly seen?
Incidence: rare in the jaws 6-8% occur in gnathic skeleton Age: 3-4th decade (mean age 33 years) Gender: male > female Site: mandible = maxilla, paranasal sinuses, skull
55
What are the typical symptoms of osteosarcoma of head and neck?
Painful swelling Parasthesia Loose teeth
56
What are the radiographic findings of osteosarcoma?
Mixed radiolucent/radiopaque Destructive Poorly defined infiltrative borders Sunburst pattern in 25% Symmetric widening of the PDL Calcification above the level of the alveolar crest Spiking root resorption
57
What are the histological features of osteosarcoma?
Pleomorphic malignant mesenchymal cells Spindled to polygonal morphology Osteoid production Osteoblastic, chondroblastic, fibroblastic types Atypical mitotic figures Necrosis Gnathic tumors are better differentiated
58
What blood findings would be seen in osteosarcomas?
Elevated alkaline phosphatase
59
How is osteosarcoma treated?
Radical surgery Chemotherapy Radiation therapy
60
What is the prognosis of osteosarcoma like?
It is an aggressive neoplasm with 30 - 50% survival Local recurrence rate of 70% Metastasis (6-50%) Lung brain and lymph nodes Mortality is persistent with local/regional disease
61
Which tumours metastasize commonly to the bone?
Carcinomas of the: Breast Prostate Lung Kidney Thyroid
62
Which bones do tumours typically metastasize to?
Hematogenous spread to the vertebral column, pellvis, ribs and skull
63
How do metastatic tumours affect bone?
Can be osteolytic or osteoblastic (cytokine mediated)
64
What is the prognosis like in metastatic tumours?
Poor
65
How common are metastatic tumours to the jaws?
Uncommon (16%)
66
Where on the face do metastatic tumours metastasize to?
Mandible typically (80%)
67
What are the symptoms of metastatic cancer to the jaws?
Painful mass - swelling Loose teeth Parasthesia
68
What are the radiographic findigns of metastatic tumours to the jaws?
Osteolytic: ill defined destructive radiolucency Osteoblastic: radiopaque or mixed lesion May simulate periapical or periodontal disease
69
What are the histological features of metastatic tumours to the jaws?
Infiltrating nests and cords of pleomorphic epithelial cells with fibrous stroma
70
What is the prognosis like in metastatic tumours ot the jaws?
Poor prognosis for widely disseminated disease. Unlikely to survive 1 year.

DMD3 (53 decks)

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