fibro-osseous, giant cell lesions, other bone diseases Flashcards

1
Q

what does fibro-osseous lesion mean?

A
  • progressive replacement of bone by fibrous tissue
  • followed by its partial maturation back towards lamellar bone
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2
Q

what are the different types of fibro-osseous lesions? (3)

A
  • cemento-ossifying fibroma
  • fibrous dysplasia
  • cemento-osseous dysplasia
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3
Q

describe cemento-ossifying fibroma (what, demographic, radiograph)

A
  • benign neoplasm of mesenchymal blast cells of PDL (fibro-osseous lesion)
  • young adult females (10-40yo), occasionally children (aggressive)
    S = mandible, premolar-molar region
    S = often small
    S = irregularly rounded
    O = well-defined, thin encapsulating RL line +/- sclerotic outer rim
    R = starts RL then mixed (wispy trabeculae or speckled)
    E = displacement, bony expansion
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4
Q

cemento-ossifying fibroma common site

A

mandible, premolar-molar region

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5
Q

cemento-ossifying fibroma radiographic appearance

A

S = mandible, premolar-molar region
S = often small
S = irregularly rounded
O = well-defined, thin encapsulating RL line +/- sclerotic outer rim
R = starts RL then mixed (wispy trabeculae or speckled)
E = displacement, bony expansion

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6
Q

cemento-ossifying fibroma demographic

A
  • young adult females (10-40yo)
  • occasionally children (aggressive)
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7
Q

cemento-ossifying fibroma differential diagnosis (up to 5)

A
  • fibrous dysplasia (less well-defined)
  • cementoblastoma
  • central giant cell granuloma
  • calcifying odontogenic cyst/tumour
  • adenomatoid odontogenic tumour
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8
Q

cemento-ossifying fibroma histology features (2)

A

(not diagnostic alone)
- fibrous capsule
- trabecular or psammomatoid bone

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9
Q

cemento-ossifying fibroma treatment

A

surgical excision +/- surgical reconstruction
(recurrence unlikely)

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10
Q

describe fibrous dysplasia (what, demographic, radiographic)

A
  • mostly sporadic mutation of GNAS1 gene = dysplastic bone
  • fibro-osseous lesion
  • 0-20yo, continues until growth complete
  • may have nerve compression and neurological issues
    S = posterior maxilla and zygomatic process, monostotic
    S =
    S =
    O = ill-defined margins, gradually blends with adjacent normal bone
    R = RL initially, homogenous ground glass appearance once mature
    E = displacement, hard to see lamina dura, rarely root resorption, jaw expansion and cortical thinning
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11
Q

what is Albright’s syndrome?

A

polyostotic fibrous dysplasia with skin and endocrine abnormalities

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12
Q

fibrous dysplasia demographic

A

0-20yo (continues until growth is complete)

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13
Q

fibrous dysplasia common site

A

posterior maxilla and zygomatic process (usually monostotic)

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14
Q

fibrous dysplasia radiographic appearance

A

S = posterior maxilla and zygomatic process, monostotic
S =
S =
O = ill-defined margins, gradually blends with adjacent normal bone
R = RL initially, homogenous ground glass appearance once mature
E = displacement, hard to see lamina dura, rarely root resorption, jaw expansion and cortical thinning

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15
Q

fibrous dysplasia histology (3)

A

(not diagnostic alone)
- cellular fibrous tissue containing woven bone islands/trabeculae in S/C/Y shapes (“Chinese characters”)
- gradual maturation to lamellar bone
- merges with surrounding normal bone (no defined margin)

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16
Q

what is the main radiographic difference between cemento-ossifying fibroma and fibrous dysplasia?

A

outline - cemento-ossifying fibroma has radiolucent rim, fibrous dysplasia merges

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17
Q

describe cemento-osseous dysplasia briefly (what, demographic, site, histology)

A
  • fibro-osseous lesion
  • likely from undifferentiated cells of PDL
  • 3 main types = periapical, focal, florid
  • young-middle aged Afrocaribbean or Asian females
  • found in tooth bearing areas
  • histology not diagnostic = gradual maturation of fibrous tissue to sclerotic dense bone (few blood vessels), “ginger root” appearance of trabeculae
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18
Q

describe periapical cemento-osseous dysplasia (demographic, radiograph)

A
  • young female Afrocaribbean (20yo+)
    S = affects PA area of several VITAL teeth, lower 1/6s
    S =
    S =
    O =
    R = starts as focus of fibrous tissue replacement (RL) –> central radiopacity appears –> radiopaque with RL rim (mature)
    E = asymptomatic, no obvious swelling
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19
Q

periapical cemento-osseous dysplasia management

A

no treatment
periodic follow up to assess prognosis

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20
Q

describe focal cemento-osseous dysplasia (2)

A
  • periapical cemento-osseous dysplasia seen as a single lesion in one location (same features otherwise)
  • may persist in bone if tooth has been extracted
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21
Q

describe florid cemento-osseous dysplasia (demographic, symptom/pain, radiograph)

A
  • middle aged female Afrocaribbean and Asian
  • possibly low-grade intermittent poorly localised discomfort
    S = multiquadrant, vital teeth; bilateral, mandible
    S =
    S =
    O =
    R = RL –> mixed with RL periphery –> amorphous dense radiopaque area with thin RL margin
    E = may cause jaw expansion, displacement, hypercementosis of associated teeth
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22
Q

possible complications of florid cemento-osseous dysplasia (2)

A
  • prone to infection after XTN (poor blood supply)
  • late stage = infection and osteomyelitis
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23
Q

describe central giant cell granuloma (symptoms, demographic, radiograph)

A
  • benign, idiopathic
  • may be slow/painless or aggressive (discomfort, tenderness, paraesthesia)
  • young <20yo
    S = anterior to 6 in mandible (or anterior maxilla); posterior in older pts
    S =
    S = unilocular, irregularly rounded/ovoid
    O = well-defined, often lacking cortication
    R = RL –> ill -defined wispy septa, honeycomb
    E = jaw expansion, displacement, root resorption
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24
Q

central giant cell granuloma treatment

A

surgical curettage +/- denosumab
(more aggressive lesions = high risk of recurrence)

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25
Q

central giant cell granuloma demographic

A

<20yo (80%)

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26
Q

central giant cell granuloma common site

A
  • mandible, anterior to 6s
  • posterior in older pts
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27
Q

central giant cell granuloma radiographic appearance

A

S = anterior to 6 in mandible (or anterior maxilla); older pts = posterior
S =
S = unilocular, irregularly rounded/ovoid
O = well-defined, often lacking cortication
R = RL –> ill -defined wispy septa, honeycomb
E = jaw expansion, displacement, root resorption

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28
Q

histological features of giant cell lesion (4)

A
  • multinucleate giant cells, aggregated into lobules, separated by fibrous bands
  • may have thin bone within septae
  • in a background of connective tissue stroma
  • vascular and lots of haemorrhage
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29
Q

what gives giant cell granulomas their colour?

A

brown - haemosiderin from RBC breakdown (vascular)

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30
Q

describe aneurysmal bone cyst (what, types, demographic, radiographic, histology)

A
  • benign but highly destructive tumour-like lesion
  • 2 types = primary (gene translocation) or secondary (with other bone lesions)
  • young females <30yo
  • uncommon in jaws
  • rapidly growing bony swelling with mild pain
    S = posterior mandible/ramus
    S = variable
    S = uni or multilocular
    O = well-defined
    R = RL with faint wispy septae (soap bubble)
    E = tooth displacement
  • blood-filled spaces separated by connective tissue septae containing trabeculae/osteoid tissue
31
Q

aneurysmal bone cyst histology

A

blood-filled spaces separated by connective tissue septae containing trabeculae/osteoid tissue

32
Q

aneurysmal bone cyst radiographic appearance (oral)

A

S = posterior mandible/ramus
S = variable
S = uni or multilocular
O = well-defined
R = RL with faint wispy septae (soap bubble)
E = tooth displacement

33
Q

aneurysmal bone cyst common site

A

mainly long bones
posterior mandible/ramus if in jaws (2%)

34
Q

aneurysmal bone cyst demographic

A

young females <30yo

35
Q

types of aneurysmal bone cyst

A
  • primary (gene translocation)
  • secondary (with other bone lesions)
36
Q

central giant cell granuloma differential diagnosis (2)

A
  • aneurysmal bone cyst
  • hyperparathyroidism (brown tumours)
37
Q

describe hyperparathyroidism briefly (types, demographic, symptoms, radiographic general and oral)

A
  • primary (gland disorder) or secondary (low serum calcium)
  • middle aged female (30-60yo)
  • stones, bone (decreased density), groans, thrones, psychiatric overtones
    S = hands/fingers initially then generalised osteopenia, brown tumours in mandible
    O = well-defined tumours
    R = ground glass bone, soap bubble tumours
    E = demineralisation and thinning of cortices, brown tumours cause displacement and expansion
38
Q

hyperparathyroidism systemic radiographic differential diagnoses (2)

A
  • renal osteodystrophy
  • post-menopausal osteoporosis
39
Q

hyperparathyroidism brown tumours more common with which type?

A

secondary hyperparathyroidism

40
Q

hyperparathyroidism demographic

A

middle aged females (30-60yo)

41
Q

describe cherubism (what, age, radiograph)

A
  • inherited AD disorder, bone replaced with fibrous tissue giving chubby cheeked appearance with “upwards gaze”
  • early childhood 2-6yo, stabilises during puberty
  • may affect breathing
    S = bilateral, mandible +/- maxilla (starts in posterior mandible), midline if severe
    S =
    S = multilocular
    O =
    R = RL
    E = painless enlargement, delayed eruption
42
Q

cherubism age

A

children 2-6yo, stabilises during puberty

43
Q

cherubism radiographic appearance

A

S = bilateral, mandible +/- maxilla
- starts in posterior mandible (rarely in condyle), midline if severe
S =
S = multilocular
O =
R = RL
E = painless enlargement, delayed eruption

44
Q

describe Paget’s disease (what, age, symptoms, radiograph)

A
  • disorder of bone turnover due to gene mutations +/- viral
  • > 40yo (increasing with age)
  • localised bone pain, pathological fracture, CN effects, bone deformity, tooth migration
    S = whole of selected bone, maxilla, axial skeleton
    S =
    S =
    O =
    R = radiolucent with expansion –> finely trabeculated/ground glass, loss of corticomedullary differentiation –> cotton wool
    E = osteoporosis circumscripta leads to basilar invagination, hypercementosis
45
Q

Paget’s disease possible presenting complaint (5)

A
  • localised bone pain
  • pathological fracture
  • CN effects
  • bone deformity
  • tooth migration
46
Q

Paget’s disease age group

A

> 40yo and increasing with age

47
Q

Paget’s disease radiographic appearance

A

S = whole of selected bone, maxilla, axial skeleton
S =
S =
O =
R = radiolucent with expansion –> finely trabeculated/ground glass, loss of corticomedullary differentiation –> cotton wool
E = osteoporosis circumscripta leads to basilar invagination, hypercementosis

48
Q

describe osteoporosis circumscripta in Paget’s disease (3)

A
  • characteristic wave of RL starting from front of skull vault, working progressively backwards
  • sharp leading margin
  • cranial vault becomes thickened and softened and droops = basilar invagination/tam o’shanter skull (flattened)
49
Q

what 3 bone diseases show ground glass appearance in the jaws and how can you differentiate them?

A
  • fibrous dysplasia (localised, younger patients)
  • hyperparathyroidism (generalised, middle-aged)
  • Paget’s disease (whole bone, elderly patients, raised serum ALP)
50
Q

what investigations might you do for Paget’s disease and what will they show? (3)

A
  • biopsy if unsure
    – increased OC resorption but net increase in bone; thick trabeculae
    – fibrous marrow with spherical new bone deposition (may fuse = sclerotic bone)
  • blood tests = raised serum ALP
  • bone scintigraphy = Lincoln’s sign (monostotic)
51
Q

describe osteoma (clinical, demographic, histology)

A
  • well-demarcated, slow-growing localised growth of compact +/or cancellous bone, non-infiltrating
  • may be pedunculated, lobulated
  • mandible > maxilla
  • may arise in sinus (frontal mainly) = localised sinusitis
  • > 40yo, males
  • histologically normal bone, indistinguishable from a torus
52
Q

describe a torus (what, demographic, location, histology)

A
  • non-neoplastic overgrowth of bone, grows as the patient grows
  • starts in adolescence
  • females
  • lower lingual premolar area OR upper palatal midline (Asian, Inuit Indian Eskimo population)
  • histologically normal bone
53
Q

osteoma age group

A

> 40yo

54
Q

osteoma sites

A

mandible > maxilla
sinus (mostly frontal)

55
Q

when does a torus become evident?

A

adolescence

56
Q

torus locations

A

palate midline
lower lingual premolar

57
Q

describe Gardner syndrome (AKA, triad)

A
  • AKA familial adenomatous polyposis
  • multiple osteomas throughout body
    triad:
  • soft tissue tumours/abnormalities
  • hard tissue abnormalities
  • multiple intestinal polyposis
58
Q

Gardner syndrome triad

A
  • soft tissue tumours/abnormalities = fibroma, lipoma, neurofibroma, scar hypertrophy, epidermoid inclusion cysts
  • hard tissue abnormalities = osteomas, hyperostosis, hypercementosis, odontoma, impacted and supernumerary teeth, mandibular bony overgrowth
  • multiple intestinal polyposis (high potential for malignant change in 40s)
59
Q

describe osteosarcoma (what, age with type, radiograph)

A
  • rare, primary malignancy of bone
  • young 10-30yo = long bones, axial skeleton, aggressive
  • mid-30s = jaw lesions (mandible>)
  • small peak in old age with underlying conditions or post-irradiation sarcoma
  • often slow growth
    radiograph:
  • initially diffuse poorly defined mixed or increased radiodensity
  • whitening of PDL space (infiltration)
  • sunray spiculation of new bone
60
Q

osteosarcoma histology (4)

A
  • neoplastic bone formed by pleomorphic osteoblasts (nuclear hyperchromatism, mitoses)
  • chicken wire bone matrix
  • infiltration
  • fibroblastic, chondroblastic or angiomatous
61
Q

osteosarcoma treatment

A

surgery/resection (poor prognosis)

62
Q

osteosarcoma age and type (3)

A
  • young 10-30yo = long bones, axial skeleton, aggressive
  • mid-30s = jaw lesions (mandible>)
  • small peak in old age with underlying conditions or post-irradiation sarcoma
63
Q

osteosarcoma radiographic appearance (3)

A
  • initially diffuse poorly defined mixed or increased radiodensity
  • whitening of PDL space (infiltration)
  • sunray spiculation of new bone
64
Q

describe chondrosarcoma (what, clinical, radiograph, histology)

A
  • rare malignant bone tumour producing cartilaginous matrix
  • common in larynx or nasal cavity
  • painless, enlarging, firm +/- ulceration
    radiograph:
  • poorly defined margins (infiltrates)
  • patchy mixed radiodensity “cloud-like whirls” + sunray spiculation
  • displace or resorb teeth
    histology = unevenly spaced, disorganised multinucleate cells in cartilaginous matrix
65
Q

chondrosarcoma radiographic appearance (3)

A
  • poorly defined margins (infiltrates)
  • patchy mixed radiodensity “cloud-like whirls” + sunray spiculation
  • displace or resorb teeth
66
Q

chondrosarcoma histology

A

unevenly spaced, disorganised multinucleate cells in cartilaginous matrix

67
Q

describe multiple myeloma (what, age, symptoms, jaw and radiograph)

A
  • malignant proliferation of plasma cells
  • middle-aged to elderly
  • back/bone pain, pathological fracture, renal involvement, Bence Jones proteins
  • mandible > maxilla - punched out RL above/below IDC
68
Q

multiple myeloma age group

A

middle-aged to elderly

69
Q

multiple myeloma radiographic appearance in jaws

A
  • mandible > maxilla
  • punched out RL above/below IDC
70
Q

multiple myeloma histology (3)

A
  • bone destruction, replacement with soft fleshy tissue
  • confluent mass of plasma-like cells, monoclonal population stained with light chains (“light chain restriction”)
  • cytological features of malignancy
71
Q

describe Langerhans cell histiocytosis (what, types with age/lesions)

A
  • spectrum of diseases - neoplasm-like proliferation of Langerhans cells/histiocytes
  • Letterer-Siwe disease (infancy, visceral and skin lesions)
  • Hand-Schuller-Christian disease (childhood, bone lesions)
  • eosinophilic granuloma (older children/adults, bone lesions)
72
Q

Langerhans cell histiocytosis radiographic appearance (2)

A
  • punched out radiolucency
  • above or below IDC
73
Q

Langerhans cell histiocytosis histology (4)

A
  • mass of uniform large cells with pale cytoplasm and kidney bean nuclei (resemble Langerhans cells without dendrites)
  • variable number of eosinophils (bilobed nucleus)
  • occasional necrosis
  • S100+ and CD1a+ for diagnosis