fibro-osseous, giant cell lesions, other bone diseases Flashcards
what does fibro-osseous lesion mean?
- progressive replacement of bone by fibrous tissue
- followed by its partial maturation back towards lamellar bone
what are the different types of fibro-osseous lesions? (3)
- cemento-ossifying fibroma
- fibrous dysplasia
- cemento-osseous dysplasia
describe cemento-ossifying fibroma (what, demographic, radiograph)
- 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
cemento-ossifying fibroma common site
mandible, premolar-molar region
cemento-ossifying fibroma radiographic appearance
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
cemento-ossifying fibroma demographic
- young adult females (10-40yo)
- occasionally children (aggressive)
cemento-ossifying fibroma differential diagnosis (up to 5)
- fibrous dysplasia (less well-defined)
- cementoblastoma
- central giant cell granuloma
- calcifying odontogenic cyst/tumour
- adenomatoid odontogenic tumour
cemento-ossifying fibroma histology features (2)
(not diagnostic alone)
- fibrous capsule
- trabecular or psammomatoid bone
cemento-ossifying fibroma treatment
surgical excision +/- surgical reconstruction
(recurrence unlikely)
describe fibrous dysplasia (what, demographic, radiographic)
- 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
what is Albright’s syndrome?
polyostotic fibrous dysplasia with skin and endocrine abnormalities
fibrous dysplasia demographic
0-20yo (continues until growth is complete)
fibrous dysplasia common site
posterior maxilla and zygomatic process (usually monostotic)
fibrous dysplasia radiographic appearance
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
fibrous dysplasia histology (3)
(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)
what is the main radiographic difference between cemento-ossifying fibroma and fibrous dysplasia?
outline - cemento-ossifying fibroma has radiolucent rim, fibrous dysplasia merges
describe cemento-osseous dysplasia briefly (what, demographic, site, histology)
- 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
describe periapical cemento-osseous dysplasia (demographic, radiograph)
- 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
periapical cemento-osseous dysplasia management
no treatment
periodic follow up to assess prognosis
describe focal cemento-osseous dysplasia (2)
- periapical cemento-osseous dysplasia seen as a single lesion in one location (same features otherwise)
- may persist in bone if tooth has been extracted
describe florid cemento-osseous dysplasia (demographic, symptom/pain, radiograph)
- 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
possible complications of florid cemento-osseous dysplasia (2)
- prone to infection after XTN (poor blood supply)
- late stage = infection and osteomyelitis
describe central giant cell granuloma (symptoms, demographic, radiograph)
- 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
central giant cell granuloma treatment
surgical curettage +/- denosumab
(more aggressive lesions = high risk of recurrence)
central giant cell granuloma demographic
<20yo (80%)
central giant cell granuloma common site
- mandible, anterior to 6s
- posterior in older pts
central giant cell granuloma radiographic appearance
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
histological features of giant cell lesion (4)
- 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
what gives giant cell granulomas their colour?
brown - haemosiderin from RBC breakdown (vascular)
describe aneurysmal bone cyst (what, types, demographic, radiographic, histology)
- 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
aneurysmal bone cyst histology
blood-filled spaces separated by connective tissue septae containing trabeculae/osteoid tissue
aneurysmal bone cyst radiographic appearance (oral)
S = posterior mandible/ramus
S = variable
S = uni or multilocular
O = well-defined
R = RL with faint wispy septae (soap bubble)
E = tooth displacement
aneurysmal bone cyst common site
mainly long bones
posterior mandible/ramus if in jaws (2%)
aneurysmal bone cyst demographic
young females <30yo
types of aneurysmal bone cyst
- primary (gene translocation)
- secondary (with other bone lesions)
central giant cell granuloma differential diagnosis (2)
- aneurysmal bone cyst
- hyperparathyroidism (brown tumours)
describe hyperparathyroidism briefly (types, demographic, symptoms, radiographic general and oral)
- 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
hyperparathyroidism systemic radiographic differential diagnoses (2)
- renal osteodystrophy
- post-menopausal osteoporosis
hyperparathyroidism brown tumours more common with which type?
secondary hyperparathyroidism
hyperparathyroidism demographic
middle aged females (30-60yo)
describe cherubism (what, age, radiograph)
- 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
cherubism age
children 2-6yo, stabilises during puberty
cherubism radiographic appearance
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
describe Paget’s disease (what, age, symptoms, radiograph)
- 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
Paget’s disease possible presenting complaint (5)
- localised bone pain
- pathological fracture
- CN effects
- bone deformity
- tooth migration
Paget’s disease age group
> 40yo and increasing with age
Paget’s disease radiographic appearance
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
describe osteoporosis circumscripta in Paget’s disease (3)
- 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)
what 3 bone diseases show ground glass appearance in the jaws and how can you differentiate them?
- fibrous dysplasia (localised, younger patients)
- hyperparathyroidism (generalised, middle-aged)
- Paget’s disease (whole bone, elderly patients, raised serum ALP)
what investigations might you do for Paget’s disease and what will they show? (3)
- 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)
describe osteoma (clinical, demographic, histology)
- 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
describe a torus (what, demographic, location, histology)
- 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
osteoma age group
> 40yo
osteoma sites
mandible > maxilla
sinus (mostly frontal)
when does a torus become evident?
adolescence
torus locations
palate midline
lower lingual premolar
describe Gardner syndrome (AKA, triad)
- AKA familial adenomatous polyposis
- multiple osteomas throughout body
triad: - soft tissue tumours/abnormalities
- hard tissue abnormalities
- multiple intestinal polyposis
Gardner syndrome triad
- 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)
describe osteosarcoma (what, age with type, radiograph)
- 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
osteosarcoma histology (4)
- neoplastic bone formed by pleomorphic osteoblasts (nuclear hyperchromatism, mitoses)
- chicken wire bone matrix
- infiltration
- fibroblastic, chondroblastic or angiomatous
osteosarcoma treatment
surgery/resection (poor prognosis)
osteosarcoma age and type (3)
- 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
osteosarcoma radiographic appearance (3)
- initially diffuse poorly defined mixed or increased radiodensity
- whitening of PDL space (infiltration)
- sunray spiculation of new bone
describe chondrosarcoma (what, clinical, radiograph, histology)
- 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
chondrosarcoma radiographic appearance (3)
- poorly defined margins (infiltrates)
- patchy mixed radiodensity “cloud-like whirls” + sunray spiculation
- displace or resorb teeth
chondrosarcoma histology
unevenly spaced, disorganised multinucleate cells in cartilaginous matrix
describe multiple myeloma (what, age, symptoms, jaw and radiograph)
- 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
multiple myeloma age group
middle-aged to elderly
multiple myeloma radiographic appearance in jaws
- mandible > maxilla
- punched out RL above/below IDC
multiple myeloma histology (3)
- 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
describe Langerhans cell histiocytosis (what, types with age/lesions)
- 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)
Langerhans cell histiocytosis radiographic appearance (2)
- punched out radiolucency
- above or below IDC
Langerhans cell histiocytosis histology (4)
- 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
