Odontogenic Tumours Flashcards

1
Q

What is a tumour

A

A swelling that includes malignant and benign neoplasms, hamartomas and some dysplasias

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2
Q

Which tissues can neoplasms and other tumours of the jaw derive from and what does this mean?

A
  1. Can derive from odontogenic epithelium (dental lamina, reduced enamel epithelium, rests of Serres, rests of Malassez)
    - No organised dental tissue can be formed due to no signalling from mesenchyme
  2. Can derive from products of odontogenic mesenchyme (dental follicle, dental papilla, pdl)
  3. Or both
    - Signalling from mesenchyme is present, tissue capable of producing dental hard tissue
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3
Q

What is the simplified classification of Odontogenic tumours

A
  1. Benign epithelial tumours
    - Ameloblastoma
    - Calcifying epithelial odontogenic tumour (Pindborg Tumour)
    - Adenomatoid odontogenic tumour
  2. Benign mixed epithelial and mesenchymal tumours
    - Ameloblastic fibroma
    - Odontome (complex & compound)
    - Calcifying odontogenic cyst (Dentinogenic ghost cell tumour)
  3. Benign mesenchymal tumours
    - Odontogenic fibroma
    - Odontogenic myxoma/myxofibroma
    - Cementoblastoma
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4
Q

What features need to be taken into account when deciding what the lesion is?

A
  • Age and sex of the patient
  • Site or anatomical postion
  • Size
  • Shape - (unilocular/multilocular)
  • Outline
  • Relative radiodensitry and internal structure
  • Effect on adjacent surrounding structures
  • Time present - if possible
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5
Q

Alternative reasons for similar radiopaque, mixed or radiolucent lesions

A
  • Localised infection
  • Spreading infection of bone (e.g. osteomyelitis)
  • Trauma
  • Cysts
  • Tumours (non-odontogenic or odontogenic)
  • Giant cell lesions
  • Early stages of Fibro osseous lesions
  • Idiopathic lesions
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6
Q

What are the key features of an ameloblastoma

A
  • Benign neoplasm of odontogenic epithelium
  • Most common type and the most common neoplasm of the jaw
  • Usually presents between 30-50yrs
  • Most commonly forms in posterior mandible
  • Symptomless until swelling is noticed (expansion may be both lingual and buccal)
  • Ameloblastoma should be included in the differential diagnosis for nay radiolucency int he posterior alveolus and lower ramus of mandible
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7
Q

What are the radiographic features of an ameloblastoma

A
  • Rounded cyst-like, radiolucent areas with well-defined margins
  • The smallest appear unilocular
  • Larger ameloblastomas may comprise a few large clustered cysts (‘soap bubbled’ multi-locularity) or numerous small cysts a few mm across (‘honeycomb’ Multilocular pattern) or a mixture of patterns
  • Other Multilocular lesions may mimic ameloblastoma radiographically: odontogenic keratocyst, giant-cell granuloma and Odontogenic myxoma
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8
Q

What is the pathology of ameloblastomas

A
  • Cause is unknown: V600E mutation activates MAP kinase pathway which drives cell division and differentiation
  • They are a mixture of solid neoplasm and cysts, and either component may predominate
  • Solid areas comprise fibrous tissue containing islands of interconnected strands and sheets of epithelium with a peripheral layer of palisaded preameloblast-like cells
    1. Follicular type: isolated islands in connective tissue stroma
    2. Plexiform: pattern of epithelium form interlacing strand of epithelial cells within sparsely cellular connective tissue (CT is in the islands)
  • Cyst formation is common, and there are usually several large cysts as large as a few cm in diameter
  • Islands of ameloblastoma can extend into the medullary spaces of surrounding bone. Not expected in a benign neoplasm because it resembles infiltration by a malignant neoplasm. If left behind after surgery, it will seed recurrence
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9
Q

Pooja what is the behaviour of ameloblasts?

A
  • Enlarge the jaw slowly, displacing and often resorbing tooth roots, perforation the cortical bone and, if large, expanding into soft tissue constrained only by the periosteum (generally expands
  • Although benign, it can be difficult to eradicate
  • Maxillary ameloblastomas are particularly dangerous because bones are considerably thinner than the mandible. Can invade the cranium and brain and can be lethal
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10
Q

What is the treatment of ameloblasts?

A
  • Attempt to treat conservatively to avoid the morbidity of large surgical excisions, especially in adolescents - but case selection important and there may be recurrence. Small mandibular lesions can sometimes be enucleated, the cavity curated
  • Typically tx: wide surgical excision, preferably removing 10mm of apparently normal bone around the margin to ensure that any extension into the medullary bone is removed
    1. Complete excision of a large ameloblastoma may therefore require partial resection of the jaw, often with the condyle an bone grafting
    2. Smaller lesions may be excised, leaving border of jaw intact
    2. Bony repair then causes much of the jaw to re-form
  • Regular RAD follow up is essential
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11
Q

What are the clinical features of a unicystic ameloblastoma?

A
  • An ameloblastoa that has a single cyst cavity
  • Present at a younger age than conventional ameloblastoma (20-30y)
  • May account for 10% of all ameloblastomas
  • May present in a true dentigerous relationship to an unerupted third molar (may simulate any odontogenic cyst but root resorption, cortical perforation or large size may give clues and if lesion attaches at CEJ then more confident of cyst)
  • In theory, single cyst structure could mean that these could be treated by simple enucleation with low recurrence risk (as there is no extension into medullary bone) but preoperative diagnosis is difficult (can only tell unicystic from multicystic/solid conventional histologically) - often mistaken for a mural conventional ameloblastoma (which appears unicystic radiographically and requires excision not enucleation)
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12
Q

What are the clinical features of Calcifying Epithelial Odontogenic Tumour (Pindborg Tumour)

A
  • Rare neoplasm of odontogenic epithelium (benign)
  • Usually presents between 40-70y
  • Most commonly forms in posterior mandible
  • Solid tumour
  • Well defined radiolucent initially, becoming mixed radiolucency with time (increasing radiopacity when it mineralises)
  • Histopathology can resemble carcinoma
  • The only odontogenic tumour to contain amyloid (in CT; secreted by epithelial cells; mineralises)
  • Locally infiltrative like ameloblastoma
  • Treated by excision with small margin
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13
Q

Radiology of CEOT (Pindborg tumour)

A
  • Site: posterior of mandible, occasionally in maxilla
  • Size: tend to remain small
  • Shape: unilocular or multilocular
  • Outline: Variable but generally well defined and corticated
  • Relative radiolucency: Early lesions radiolucent but as tumour matures and calcifies can have areas of varying radiolucency
  • Effects on adjacent structures: Expands buccal bone
  • Differential diagnosis: Dentigerious cyst (distinguished by calcifications), odontomes
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14
Q

Radiology of unicystic ameloblastoma

A
  • Site: posterior of mandible
  • Size: several CM
  • Shape: unilocular
  • Outline: well defined and corticated
  • Relative radiolucency: radiolucent
  • Effects on adjacent structures: Displaces teeth, expands bone, resorbs roots
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15
Q

Radiology of multicystic ameloblastoma

A
  • Site: posterior of mandible
  • Size: Several CM
  • Shape: multilocular
  • Outline: well defined and corticated
  • Relative radiolucency: Radiolucent
  • Effects on adjacent structures: Expands bone, resorbs roots, displaces teeth
  • Differential diagnosis: Odontogenic keratocyst (but OKCs do not displace teeth)
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16
Q

Histology of CEOT (Pindborg tumour)

A
  • Non cystic
  • Sheaths of squamous epithelial cells in a CT stroma
  • Gross variation in nuclei size therefore cane mistaken for a poorly differentiated carcioma
  • Formation of amyloid-like material with homogenous hyaline (glassy) appearance
  • This can calcify to form concentric (Liesegang) rings
17
Q

What are the clinical features of an Adenomatoid odontogenic tumour

A
  • Benign harmatoma of odontogenic neoplasm (not a neoplasm)
  • Usually presents 15-30y, F>M
  • Most develop in anterior maxilla and form a slow growing swelling resembling a dentigerous or radicular cyst or are chance findings in the follicle of an extracted unerupted tooth
  • When in the wall of a cyst, a subtle radiographic clue is fine speckled mineralisation
  • Encapsulated
  • Treated by enucleation (non-infiltrative)
  • Histological analysis needed for true diagnosis
  • Presence of duct-like structures histologically (hence name) and mineralisation of enamel matrix like structures can occur in ‘duct’ spaces as well as dentinoid tissue (unmineralised)
18
Q

What is the radiology of adenomatoid odontogenic tumour

A
  • Site: Anterior maxilla, associated with displaced, unerupted tooth
  • Size: several CM
  • Shape: unilocular
  • Outline: well defined and corticated
  • Relative radiolucency: Non-uniformly radiolucent due to calcifications
  • Effects on adjacent structures: Expands bone and tooth displacement
  • Differential diagnosis: Dentigerious cyst (distinguished by calcifications)
19
Q

Clinical features of an ameloblastic fibroma

A
  • Rare
  • Neoplasm of both odontogenic epithelium and mesenchyme (but lacking mineralisation and dental hard tissues as ameloblast-like cells are non-induced by ectomesenchyme)
  • Usually seen in children or young adults
  • Can be very destructive in the growing facial bones
  • Usually posterior mandible; expands jaw slowly and displaces teeth or prevents eruption
  • Solid lesion but appears as unilocular or Multilocular radiolucency
  • Treated by excision with small margin
  • Can undergo malignant change (following incomplete removal)
  • Histology is branching strands/islands of odontogenic epithelium (ameloblast like cells) in ectomesencymal stroma
20
Q

Radiology of ameloblastic fibroma

A
  • Site: posterior of mandible mainly but can occur in maxilla
  • Size: several CM
  • Shape: unilocular or multilocular
  • Outline: corticated margins
  • Relative radiolucency: uniformly radiolucent
  • Effects on adjacent structures: Bony expansion and tooth resorption
  • Differential diagnosis: ameloblastoma (Distinguished by age of incidence)
21
Q

Clinical features of calcifying odontogenic cyst

A
  • Solid and cystic variants (solid lesions are more aggressive)
  • About 10% associated with odontomes
  • Calcifications in cyst wall may suggest the diagnosis
  • Forms at any site in alveolar ridge, usually posteriorly
  • Diagnosed by finding ghost (no nuclei, just outline of cell) and ameloblast-like cells histologically. There is dentine-like dentinoid found
  • Treat by excision with margin of normal tissue of solid variant but cystic respond to enucleation
22
Q

Radiology of calcifying odontogenic cyst

A
  • Site: either jaw, any tooth bearing area
  • Size: varies around 4cm
  • Shape: unilocular or multilocular
  • Outline: well defined and corticated
  • Relative radiolucency: Early lesions radiolucent but as cyst matures and calcifies can have areas of varying radiolucency
  • Effects on adjacent structures: Some bony expansion, displacement of teeth, root resorption
  • Differential diagnosis: AOT, Odontome, Solid conventional ameloblastoma
23
Q

Clinical features of odontomes generally

A
  • Commonest odontogenic tumours
  • Developmental malformations (harmatomas) of dental tissues and not neoplasms
  • Chance radiographic finding or present after preventing tooth eruption (can displace teeth)
  • Develop like teeth with initial radiolucent phase, intermediate stage of mixed radiolucency, finally densely radiopaque as E+D form internally
  • Commons sites are anterior maxilla and posterior mandible
  • Benign and stop growing once mature
  • Two types: compound and complex
  • Treated by enucleation and do not recur
24
Q

Clinical features of Compound odontomes

A
  • Clusters of many separate small tooth-like structures (denticles) within one crypt, whole lesion not usually larger than 2cm
  • Usually found on anterior maxilla and causes minimal swelling
  • Histologically denticles are embedded in CT and have a fibrous capsule around the entire lesion. Each denticle has an organised pulp centrally and an enamel cap over the dentine
  • Denticles develop like normal teeth, mineralise fully and once mature stop growing
25
Q

Clinical features of complex odontomes

A
  • A single irregular mass of hard and soft dental tissues, having no morphological resemblance to a tooth and recently forming a cauliflower shaped disorganised nodule of enamel and dentine
  • May reach several cm in size and often expand the jaw
  • Radiographically, when calcification is complete, an irregular radiopaque mass is seen containing areas of densely radiopaque enamel
  • Histologically, mass consists of all dental tissues in a disordered arrangement
26
Q

What are the clinical features of an odontogenic fibroma

A
  • Benign neoplasm of fibrous tissue
  • Wide age range
  • Usually mandible
  • Slow-growing asymptomatic mass that may eventually expand the jaw
  • Sharply defined, rounded radiolucent area in a tooth-bearing region
  • Spindle shaped fibroblasts and bundles of collagen fibres (some contains rests of odontogenic epithelium)
  • Enucleated easily from surroundings bone and do not recur
27
Q

What is the radiology of an odontogenic fibroma?

A
  • Site: more common in mandible (premolar region)
  • Size: several cm
  • Shape: unilocular
  • Outline: well defined and corticated
  • Relative radiolucency: Uniformly radiolucent
  • Effects on adjacent structures: displaces teeth, resporbs roots
  • Differential diagnosis: radicular cyst (but this requires a non-vital tooth)
28
Q

What are the clinical features of an odontogenic myxoma?

A
  • Benign neoplasm
  • The third commonest odontogenic tumour after odontomes and ameloblastomas
  • Most arise 10-30y and produces symptomatic swellings of jaw (usually posterior mandible)
  • Myxomas cause radiolucent areas with scalloped indistinct margins or soap-bubble or honeycomb appearance
  • They displace teeth after destroying supporting bone and are more extensive than is seen radiographically
  • Resembles normal dental follicle histologically (bulk of myxoma is loose myxoid ground substance containing dispersed fibroblasts)
  • Grow by secretion of the ground substance by fibroblasts rather than cell proliferation
  • the gelatinous consistency allows the tumour tissue to penetrate widely between medullary bone trabeculae without a Clear margin, difficult to remove
  • Excision with margin and removal of associated teeth required
29
Q

Radiology of odontogenic myxoma

A
  • Site: posterior of mandible, may be associated with missing or unerputed tooth
  • Size: variable but can get large if untreated
  • Shape: multilocular (soap-bubble or honeycomb) but can be unilocular
  • Outline: Well defined with variable cortication
  • Relative radiolucency: Radiolucent with fine internal radiopaque septa
  • Effects on adjacent structures: Displace teeth, resort boots, extensive buccal and lingual expansion with cortical perforation if large
30
Q

Clinical features of cementoblastoma

A
  • Benign neoplasms of cementoblasts that form mass of cementum on a tooth root
  • Odontogenic
  • Distinct presentation and behaviour from osteoblastomas, even though histologically almost identical
  • Young adults <25y
  • Usually detected small, max of a few cm
  • Slow growing, sometimes painful and expand the jaw
  • Most commonly at apex of vital 6
  • Root heavily resorbed and fused to lesion
  • Radiographically: radiopaque mass with thin radiolucent margin attached to the root of a tooth. Mass may be rounded or irregular inshore and mottled in texture
  • Treated by xla of tooth and enucleation and curettage of the bony cavity
  • Recurrence is unusual but incomplete removal leads to regrowth