Oral medicine:16. radiolucencies and radio-opacities. c. odontogenic tumours

From Volume 41, Issue 3, April 2014 | Pages 274-278

Authors

David H Felix

BDS, MB ChB, FDS RCS(Eng), FDS RCPS(Glasg), FDS RCS(Ed), FRCPE

Postgraduate Dental Dean, NHS Education for Scotland

Articles by David H Felix

Jane Luker

BDS, PhD, FDS RCS, DDR RCR

Consultant and Senior Lecturer, University Hospitals Bristol NHS Foundation Trust, Bristol

Articles by Jane Luker

Crispian Scully

CBE, DSc, DChD, DMed (HC), Dhc(multi), MD, PhD, PhD (HC), FMedSci, MDS, MRCS, BSc, FDS RCS, FDS RCPS, FFD RCSI, FDS RCSEd, FRCPath, FHEA

Bristol Dental Hospital, Lower Maudlin Street, Bristol BS1 2LY, UK

Articles by Crispian Scully

Article

David H Felix
Jane Luker
Crispian Scully

Specialist referral may be indicated if the Practitioner feels:

  • The diagnosis is unclear;
  • A serious diagnosis is possible;
  • Systemic disease may be present;
  • Unclear as to investigations indicated;
  • Complex investigations unavailable in primary care are indicated;
  • Unclear as to treatment indicated;
  • Treatment is complex;
  • Treatment requires agents not readily available;
  • Unclear as to the prognosis;
  • The patient wishes this.
  • Odontogenic tumours

    Odontogenic tumours are rare, are often asymptomatic, and discovered incidentally on imaging (Table 1). They are generally slow-growing and may reach a large size before becoming symptomatic, eg:

  • Swelling, sometimes with cortical perforation. Despite some odontogenic tumours expanding rather than destroying bone, there may be local invasion of surrounding bone;
  • Pain due to secondary infection or pathological fracture.

  • BENIGNOdontogenic epithelium with mature, fibrous stroma Odontogenic carcinomas without MALIGNANTodontogenic ectomesenchyme
    Ameloblastoma, solid/multicystic type Metastasizing (malignant) ameloblastoma
    Ameloblastoma, extra-osseous/peripheral type Ameloblastic carcinoma – primary type
    Ameloblastoma, desmoplastic type Ameloblastic carcinoma – secondary type (dedifferentiated), intraosseous
    Ameloblastoma, unicystic type Ameloblastic carcinoma – secondary type (dedifferentiated), peripheral
    Squamous odontogenic tumour Primary intra-osseous squamous cell carcinoma – solid type
    Calcifying epithelial odontogenic tumour Primary intra-osseous squamous cell carcinoma derived from keratocystic odontogenic tumour
    Adenomatoid odontogenic tumour Primary intra-osseous squamous cell carcinoma derived from odontogenic cysts
    Keratocystic odontogenic tumour (KCOT) Clear cell odontogenic carcinoma
    Odontogenic epithelium with odontogenic ectomesenchyme, with or without hard tissue formation Ghost cell odontogenic carcinoma
    Ameloblastic fibroma Odontogenic sarcomas
    Ameloblastic fibrodentinoma Ameloblastoma fibrosarcoma
    Ameloblastic fibro-odontoma Ameloblastic fibrodentino and fibro-odonto sarcoma
    Odontoma (odontome)
     Odontoma, complex type
     Odontoma, compound type
    Odontoameloblastoma
    Calcifying cystic odontogenic tumour
    Dentinogenic ghost cell tumour
    Mesenchyme and/or odontogenic ectomesenchyme with or without odontogenic epithelium
    Odontogenic fibroma
    Odontogenic myxoma/myxofibroma
    Cementoblastoma

    They usually appear as well-defined corticated unilocular or multilocular radiolucencies but, unlike cysts, they are more likely to cause root resorption and buccal and lingual cortical expansion.

    The majority of odontogenic tumours are benign. Management is surgical and is dependent upon the type of tumour and varies from enucleation to resection.

    Benign odontogenic tumours

    Benign odontogenic tumours are 100 times more common than malignant ones: most (>50%) are odontomas, or ameloblastomas (around 10%).

    Ameloblastomas are significant since they may recur or metastasize. Composed of ameloblast-like epithelial cells arranged as a peripheral layer around a central area resembling stellate reticulum, two main histological types exist. The follicular type contains discrete islands (follicles) of epithelial cells: the plexiform type consists of anastomosing strands. Ameloblastomas predominate in the posterior mandible, presenting typically in third to fifth decades as a slow-growing, painless, uni-or multi-locular mass (‘soap-bubble’ appearance on imaging) (Figures 1 and 2) usually replacing a tooth and producing more buccolingual expansion and knife edge root resorption than does KCOT (but differentiation is difficult by plain radiography or CT). MRI may then help.

    Figure 1. DPT of a well-defined multilocular radiolucency extending from the LL5 to the ramus of the mandible. Note the early apical resorption of the roots of the lower second molar. Diagnosis was an ameloblastoma.
    Figure 2. PA jaws of the ameloblastoma showing extensive lingual expansion consistent with a diagnosis of a solid rather than a cystic lesion.

    Squamous odontogenic tumour is rare and usually presents as a painless swelling and radiolucency between teeth which become mobile. It may mimic periodontal disease.

    Calcifying epithelial odontogenic tumour (CEOT: Pindborg tumour) – rare, benign but aggressive. Three distinct histological features are:

  • Sheets of pleomorphic epithelial cells, in places, characterized by a clear cytoplasm (‘clear cells’);
  • Amyloid;
  • Concentric masses of calcified tissues.
  • Usually seen in mandibular premolar or molar region associated with the crown of an impacted tooth, CEOT is radiolucent with scattered calcified components.

    Adenomatoid Odontogenic Tumour – is the ‘two-thirds tumour’ – most commonly noted in the second and third decades of life and two-thirds of cases:

  • Are in females;
  • Occur in the anterior maxilla;
  • Are associated with an impacted tooth (usually canine).
  • Sheets and strands of epithelial cells are arranged as convoluted bands and tubular structures, in which ameloblast-like cells are arranged radially around a homogeneous eosinophilic material. It presents as a well-demarcated unilocular radiolucent lesion, often with punctate calcifications. It rarely recurs after excision.

    Keratocystic odontogenic tumour (KCOT) has a propensity for destruction and recurrence locally. Radiologically, KCOT usually presents as a well-defined corticated unilocular or multilocular radiolucency which enlarges through cancellous bone, giving rise to late cortical expansion, and for its size the amount of bucco-lingual expansion is small compared to other odontogenic tumours (Figures 3 and 4). The lining has a regular keratinized stratified squamous epithelium, five to eight cell layers thick and without rete pegs. Desquamated keratin is often present within the lumen and the fibrous wall is usually thin. KCOT are often multilocular, well-defined, radiolucent, usually without an associated tooth. The keratin-rich debris shows a characteristic central signal drop on MRI T2-weighted images.

    Figure 3. Sectional DPT of an odontogenic keratocyst; note the similarity in appearance to the ameloblastoma in Figure 1 but absence of apical root resorption.
    Figure 4. Section of PA jaws showing the keratocyst with little bucco-lingual expansion in relation to its size compared to the ameloblastoma in Figure 2.

    KCOT is associated with chromosome 9 patch gene mutations. Multiple KCOTs in young patients should suggest the basal cell naevus (Gorlin-Goltz) syndrome – an autosomal dominant disorder also with midface hypoplasia, frontal bossing and prognathism, falx cerebri calcification and skeletal anomalies.

    Ameloblastic fibroma consists of islands, elongated strands, or terminal buds of ameloblast-like cells and central stellate reticulum cells, surrounded by a cellular hyaline material. It is usually well-defined, pericoronal multiloculated radiolucent and associated with an impacted tooth, often in the posterior mandible.

    Odontogenic myxoma is clinically and radiographically indistinguishable from ameloblastoma.

    Odontoma (odontome) – a ‘hamartoma’ consisting of dentine and enamel, is often associated with an impacted tooth, classified as follows:

  • Compound type (Compound composite odontomes) – multiple small simple denticles embedded in fibrous connective tissue within a capsule. Multiple lesions may be seen in Gardner syndrome;
  • Complex type (Complex composite odontomes) – an irregular mass of all dental tissues.
  • Odontomes typically present during the second decade, are more common in females than males, and often in the mandibular premolar–molar region. Typically, they can behave like teeth: they can grow and tend to erupt, or may displace adjacent teeth. Failure of a tooth to erupt is usually the justifying reason for radiographic exposure which identifies the odontome.

    Cementoblastoma is a neoplasm of cementum typically seen in patients under 25 years. Usually fused to a root (typically mandibular premolar or first molar), it is a well-defined radio-opacity with a radiolucent margin. It may cause pain which responds to NSAIDs. Hypercementosis, in contrast, is smoother, less nodular, and has a thin radiolucent margin continuous with the periodontal ligament space.

    Malignant odontogenic tumours

    These are generally considered as the malignant counterparts of the benign categories (Table 2).


    Tumour type and % of odontogenic tumours Age in years Site Association with unerupted teeth Resorption of teeth Other features
    Ameloblastoma 11% 80%<40 90% mandible molar region 38% (predominantly third molar region) 39% knife edge Multilocular; angle of mandible preserved.
    Ameloblastic fibroma 2% <2040%<10 73% mandible molar/premolar 100% Rare Multilocular: less likely to destroy areas of expanded cortex than ameloblastoma.
    Ameloblastic fibro-odontoma 2% <20 Mandible = maxilla 25% anterior jaw 100% Rare Multilocular; very small lesions cause large amount of tooth displacement.
    Adenomatoid odontogenic tumour 3% 5–50 Average 16 70% teenage 75% maxilla 90% in canine incisor region 74% Rare Snowflake opacities evenly arranged throughout lesion.
    Calcifying epithelial odontogenic tumour 1% 30–50 peak 40 Mandible 2x maxillaMolar 3x premolar 52% rarely impacted Calcification begins May occur knife edge Maybe multilocular: lesion tends to extend into body rather than ramus. Less well defined than ameloblastoma.
    Calcifying cystic odontogenic Tumour 2% 10–19 Mandible = maxilla 75% ant to 6 20–25% Occasionally Hydraulic bone expansion, expanded bone may appear perforated.
    Keratocystic odontogenic tumour Commonest odontogenic tumour 3–10% of cyst like lesions 20–50 Mandible 3x maxilla 10% multiple. CT scan if sinus involved.Radiolucency in ramus not contacting any teeth most likely keratocyst.Minimal tooth displacement.Perforation of cortex may occur.
    Odontogenic fibro/myxoma 3–5% Mean 25–35 Mandible 3x maxilla Molar>premolar rare ramus 5% often congenitally absent tooth Occasionally May be multilocular; May destroy angle of mandibleSepta intersect at 90º; may cross midline.
    Cement-ossifying fibroma 40% of FCOL 15–50 80% mandible premolar and molar Maxilla, zygoma and canine May occur Expansion of bone equally in all directions Cortex remains intact Bowing of inferior border parallels tumour mass.
    Benign cementoblastoma 9% of FCOL <25 85% mandible60% 1st molar20–25% premolars 50% resorption of fusion Only cemental lesion to be attached to root of involved tooth.
    Odontomes 67% <20 Compound: Anterior maxilla 62%. Complex: Mandible 1st 2nd molar 70% 48% Rare Compound 2x as common as complex.

    Some useful points with regard to odontogenic tumours are given in Table 3.


    Normal follicle space 2.5 mm intra-oral 3 mm DPT >2.5 80% cystic The canine has larger follicle space
    Radicular cyst or granuloma Diameter >1.6 cm or Area 200 mm
    Dentigerous cysts Lingual expansion rare
    Keratocystic odontogenic tumour 10% multiple CT scan if sinus involved. Radioluency in ramus not contacting any teeth most likely keratocyst. Minimal tooth displacement. Perforation of cortex may occur.
    Nasopalatine cyst Normal size of nasopalatine duct 6 x 7 mm 4:1 M:F Diameter >1cm ?cystic Normal well-defined lateral walls only
    Central Giant Cell Granuloma F:M 2:1 60% < 20 years of age. Associated with Paget's disease. Rare in ramus; usually anterior to first molar; uneven buccolingual expansion.
    Aneurysmal bone cyst 90% < 30 years. Cortex remains intact even when large. Marked buccolingual expansion compared to AP.
    Central haemangioma Premature exfoliation of 1º and delayed eruption of 2º teeth. Phleboliths. Vertical expansion important feature
    Arteriovenous malformation Cortical thinning without perforation Sunray appearance: Phleboliths
    Solitary bone cysts 50% > 3 cm. Few extend into ramus 70% have scalloped upper margin
    Ameloblastoma The Great Mimicker 80% < 40 years. 10–15% unilocular38% associated with unerupted teethMRI better for recurrence high signal on T2 weighted imagesAngle usually preservedLocules larger centrally
    Ameloblastic fibroma Even when small causes buccolingual expansion
    Ameloblastic odontofibroma Small lesion large tooth displacement
    Adenomatoid odontogenic tumour 74% unerupted tooth. 68% canines, 70% < 20 years, F:M 2:1
    Calcifying epithelial odontogenic tumour Frequently scalloped margin. Variable definition. Expands into body rather than ramus.
    Calcifying cystic odontogenic tumour Hydrostatic expansion. Cortical perforation 96% unilocular
    Odontogenic myxoma Soap bubble appearance; expansion and perforation May destroy angle
    Benign cementoblastoma Attaches to tooth rootOcclusal film sunray appearance
    Cement-ossifying fibroma Expansion inferior cortex of the mandible