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
Q

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

A

Benign fibro-osseus lesion

Cellular spindled fibrosis

Collagen fibers

Globular cementum matrix material

Woven osseous matrix material

Vascular channels

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26
Q

How is focal cemento-osseus dysplasia treated?

A

Curettage (Biopsy)

Watching and following up periodically.

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27
Q

What is the prognosis of focal cemento-osseus dysplasia like?

A

Excellent prognosis (limited growth potential)

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28
Q

What is florid cemento-osseus dysplasia?

A

Benign non-neoplastic dysplastic process

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29
Q

What are the radiographic findings for florid cemento-osseus dysplasia?

A

Irregular lobular dense
radiopacities

Mixed radiolucent and
radiopaque areas

Diffuse involvement
of maxilla and mandible

Bilateral

Symmetrical

30
Q

What are the histolopathologic findings of florid cemento-osseus dysplasia?

A

Fibroblastic proliferation

Dense sclerotic calcific masses

Cementum matrix

Woven bone

Inflammatory infiltrate

Simple bone cysts

31
Q

How is florid cemento-osseus dysplasia managed?

A

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
Q

What is a cementoblastoma?

A

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
Q

Which teeth are often affected by cementoblastomas?

A

Usually a mandibular premolar or first molar

34
Q

What are the clinical features of a cementoblastoma?

A

Tender, sometimes painful swelling

Located on the buccal and lingual/palatal aspect of the alveolus

35
Q

Who typically gets a cementoblastoma?

A

Under 30 years of age

M:F 1.2:1

36
Q

Which tooth is most commonly affected by a cementoblastoma?

A

Mandibular 1st molar

80% of them are in the mandible

37
Q

What are the radiographic features of a cementoblastoma?

A

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
Q

What are the histological features of a cementoblastoma?

A

Sheets or trabeculae of cementum-like calcified tissue with variably prominent reversal lines, sometimes with a pagetoid appearance

39
Q

How is cementoblastoma treated?

A

Surgery

Recurrence 35 - 60%

40
Q

How are odontogenic tumours classified?

A

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
Q

What benign tumours fall under the category of odontogenic epithelium without odontogenic ectomesenchyme?

A

Ameloblastoma

Squamous odontogenic tumour

Calcifying epithelial odontogenic
tumour (Pindborg Tumour)

42
Q

What benign tumours fall under the category of odontogenic epithelium without odontogenic ectomesenchyme +/- dental hard tissue?

A

Ameloblastic fibroma

Ameloblastic fibro-dentinoma

Ameloblastic fibro-odontome

Adenomatoid odontogenic tumour

Calcifying odontogenic cyst

Complex odontome

Compound odontome

43
Q

What benign tumours fall under the category of odontogenic ectomesenchyme +/- odontogenic epithelium?

A

Odontogenic fibroma

Myxoma [myxofibroma]

Benign cementoblastoma (‘true’ cementoma)

44
Q

Which odontogenic tumours are malignant?

A

Clear cell odontogenic carcinoma

Malignant ameloblastoma

Primary intra-osseus carcinoma

Malignant variants of other odontogenic epithelial tumours

Malignant changes in odontogenic cysts

45
Q

What are the clinical features of ameloblastoma?

A

Slow growing, polycystic or monocystic

46
Q

Who typically gets ameloblastoma?

A

More males after the age of 40

47
Q

What is an osteoma?

A

A reactive, developmental (non-neoplastic) bone forming tumour

48
Q

Where is an osteoma typically found?

A

Craniofacial bones: Skull and paranasal sinuses

49
Q

What are the symptoms of an osteoma?

A

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
Q

What is an osteosarcoma?

A

Malignant mesenchymal neoplasm

Osteoid production by tumor cells

Most common primary bone malignancy

51
Q

What is the most common age of onset of an osteosarcoma? Is it male or female dominant?

A

2nd decade of life

More common in males Male:Female 1.6:1

52
Q

What are the most common sites for an osteosarcoma?

A

Metaphysis of long bones

Distal femus, proximal tibia, humerus

53
Q

What could potentially cause osteosarcoma?

A

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
Q

Where on the head and neck is osteosarcome most commonly seen?

A

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
Q

What are the typical symptoms of osteosarcoma of head and neck?

A

Painful swelling

Parasthesia

Loose teeth

56
Q

What are the radiographic findings of osteosarcoma?

A

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
Q

What are the histological features of osteosarcoma?

A

Pleomorphic malignant mesenchymal cells

Spindled to polygonal morphology

Osteoid production

Osteoblastic, chondroblastic, fibroblastic types

Atypical mitotic figures

Necrosis

Gnathic tumors are better differentiated

58
Q

What blood findings would be seen in osteosarcomas?

A

Elevated alkaline phosphatase

59
Q

How is osteosarcoma treated?

A

Radical surgery

Chemotherapy

Radiation therapy

60
Q

What is the prognosis of osteosarcoma like?

A

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
Q

Which tumours metastasize commonly to the bone?

A

Carcinomas of the:

Breast

Prostate

Lung

Kidney

Thyroid

62
Q

Which bones do tumours typically metastasize to?

A

Hematogenous spread to the vertebral column, pellvis, ribs and skull

63
Q

How do metastatic tumours affect bone?

A

Can be osteolytic or osteoblastic (cytokine mediated)

64
Q

What is the prognosis like in metastatic tumours?

A

Poor

65
Q

How common are metastatic tumours to the jaws?

A

Uncommon (16%)

66
Q

Where on the face do metastatic tumours metastasize to?

A

Mandible typically (80%)

67
Q

What are the symptoms of metastatic cancer to the jaws?

A

Painful mass - swelling

Loose teeth

Parasthesia

68
Q

What are the radiographic findigns of metastatic tumours to the jaws?

A

Osteolytic: ill defined destructive radiolucency

Osteoblastic: radiopaque or mixed lesion

May simulate periapical or periodontal disease

69
Q

What are the histological features of metastatic tumours to the jaws?

A

Infiltrating nests and cords of pleomorphic epithelial cells with fibrous stroma

70
Q

What is the prognosis like in metastatic tumours ot the jaws?

A

Poor prognosis for widely disseminated disease. Unlikely to survive 1 year.