Differential diagnosis of radiolucent lesions of the jaws

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Differential diagnosis of radiolucent lesions of the jaws

Introduction

This chapter is designed to simplify the process of arriving at a radiological differential diagnosis when confronted with a radiolucency of unknown cause on a plain radiograph. This process requires clinicians to follow a methodical step-by-step approach and to know the typical features of the various possibilities. Such a step-by-step guide is suggested and summarized in Fig. 26.1. Although most lesions are still detected using plain radiographs, this process can be greatly facilitated in many cases if advanced imaging modalities, described in Chapters 16 and 18, such as computed tomography (CT), cone beam CT or magnetic resonance (MR), are available.

Unfortunately, most of the lesions encountered share several similar features and often individual conditions can present in many different ways. Thus the summary of features for the more important conditions included in this chapter is an attempt to unravel some of the inevitable confusion. Also, for simplicity, the frequency with which the various lesions present has been divided arbitrarily into common, uncommon and rare. It is hoped and intended that the reader should expand on this short-notes style framework by referring to the suggested reading list.

Step-by-step guide

Step IV

Consider the classification and subdivision of cysts and other similar radiolucencies within each of the other main disease categories, as shown in Table 26.1. This resultant list includes most of the more likely diagnostic possibilities for the unknown radiolucent lesion.

Table 26.1

Classification of the main cysts and tumours and other bone-related conditions that can present as a cyst-like radiolucency (based broadly on the 2005 WHO Classification)

Cysts  
Odontogenic Radicular (dental) cyst
  Residual radicular cyst
  Lateral periodontal cyst
  Dentigerous cyst
  Odontogenic keratocyst (keratocystic odontogenic tumour)
Non-odontogenic Nasopalatine duct / incisive canal cyst
  Bone cysts (see bone-related lesions)
Tumours and tumour-like lesions  
Benign odontogenic Ameloblastoma
(epithelial with mature, fibrous stroma without odontogenic ectomesenchyme) Squamous odontogenic tumour
Calcifying epithelial odontogenic tumour (Pindborg tumour)
  Adenomatoid odontogenic tumour
  Keratocystic odontogenic tumour (odontogenic keratocyst)
Benign odontogenic Ameloblastic fibroma
(epithelial with odontogenic ectomesenchyme, with or without hard tissue formation) Ameloblastic fibro-odontoma
Calcifying cystic odontogenic tumour (calcifying odontogenic cyst)
Benign odontogenic Odontogenic fibroma
(mesenchymal and/or odontogenic ectomesenchyme with or without odontogenic epithelium) Odontogenic myxoma
Malignant odontogenic Odontogenic carcinoma
  Odontogenic sarcoma
Non-odontogenic intrinsic primary bone tumours Benign – Fibroma
 – Chondroma
   – Central haemangioma
   – Neurofibroma
  Malignant – Osteosarcoma
   – Fibrosarcoma
   – Chondrosarcoma
Extrinsic primary tumours involving bone Squamous cell carcinoma
Secondary metastatic bone tumours  
Lymphoreticular tumours of bone Multiple myeloma
  Large cell lymphoma
  Burkitt’s lymphoma
  Ewing’s tumour
Langerhans cell disease Eosinophilic granuloma
(Histiocystosis X) Hand–Schüller–Christian disease
  Letterer–Siwe disease
Bone-related lesions  
Giant cell lesions Central giant cell lesion (granuloma)
  Brown tumour in hyperparathyroidism
  Cherubism
  Aneurysmal bone cyst
Osseous dysplasias Periapical osseous dysplasia
(Fibro-cemento-osseous lesions) Focal osseous dysplasia
(early stages) Florid osseous dysplasia
  Familial gigantiform cementoma
Other lesions Ossifying fibroma
  Fibrous dysplasia
  Simple bone cyst
  Stafne’s bone cavity

image

Step V

Compare the radiological features of the unknown radiolucency with the typical radiological features of these possible conditions. Then construct a list showing, in order of likelihood, all the conditions that the lesion might be. This list forms the radiological differential diagnosis.

Infection is described elsewhere (apical, Ch. 21, spreading, Ch. 28) and trauma is described in Chapter 29. The rest of this chapter is devoted principally to differentiating between the different cysts – the most common of the remaining categories – and the other lesions that often present as very similar radiolucencies.

Typical radiographic features of cysts

Inflammatory odontogenic cysts

Radicular (dental) cyst (Fig. 26.2)

This inflammatory cyst develops from the epithelial remnants of Hertwig’s root sheath – the cell rests of Malassez.

Note: The term buccal bifurcation cyst is used to describe an inflammatory odontogenic cyst that develops on the side of a molar tooth in relation to a buccal enamel spur or pearl.

Developmental odontogenic cysts

Lateral periodontal cyst (Fig. 26.4)

The diagnosis of this rare developmental cyst should be reserved for a cyst in the lateral periodontal region that is not an inflammatory cyst or an atypical odontogenic keratocyst. It is thought to develop from either the cell rests of the dental lamina or from remains of the reduced enamel epithelium on the lateral surface of the root.

Dentigerous (follicular) cyst (Fig. 26.5)

This cyst develops from the remnants of the reduced enamel epithelium after the tooth has formed.

• Age: Usually adolescents or young adults, 20–40-year-olds, occasionally the elderly.

• Frequency: About 20% of all cysts.

• Site: Associated with the crown of an unerupted and displaced tooth, typically teeth where eruption is impeded, e.g. image and image.

• Size: Very variable, cyst suspected if follicular space exceeds 3 mm but may grow to several centimetres in diameter and extend up into the ramus.

• Shape: —Round or oval, typically enveloping the crown symmetrically

• Outline: — Smooth

• Radiodensity: Uniformly radiolucent.

• Effects: —Associated tooth unerupted and displaced

Note: The term eruption cyst is used to describe a dentigerous cyst when it is in the soft tissues overlying the unerupted tooth.

Odontogenic keratocyst (keratocystic odontogenic tumour) (Fig. 26.6)

Somewhat controversially, in 2005 the WHO Working Group recommended that the odontogenic keratocyst be renamed the keratocystic odontogenic tumour as they felt this name better reflected its neoplastic nature. The WHO now defines this lesion as a benign uni- or multicystic intraosseous tumour of odontogenic origin with a histologically characteristic lining of parakeratinized stratified squamous epithelium with a potentially aggressive, infiltrative behaviour. It is believed to develop from the epithelium of the dental lamina – the cell rests of Serres – instead of the normal tooth which is therefore typically missing from the series. Lesions are typically solitary but multiple odontogenic keratocysts are a feature of nevoid basal cell carcinoma syndrome (Gorlin’s syndrome), which also includes multiple basal cell carcinomas, and skeletal anomalies, e.g. bifid ribs and calcification of the falx cerebri.

Non-odontogenic cysts

Nasopalatine duct/incisive canal cyst (Fig. 26.7)

This cyst develops from epithelial remnants of the nasopalatine duct or incisive canal.

• Age: Variable, but most frequently detected in middle age (40–60-year-olds).

• Frequency: Most common of all non-odontogenic cysts, affecting about 1% of total population.

• Site: Midline, anterior maxilla just posterior to the upper central incisors.

• Size: Variable, but usually from 6 mm to several centimetres in diameter.

• Shape: —Round or oval (superimposition of the nasal septum or anterior nasal spine may cause the cyst to appear heart-shaped or resemble an inverted tear drop)

• Outline: — Smooth

• Radiodensity: Uniformly radiolucent but radiopaque shadows sometimes superimposed.

• Effects: —Adjacent teeth – distal displacement, rarely resorbed

Note: Differentiation is sometimes required between a nasopalatine duct cyst and a large normal nasopalatine foramen. Several features need to be considered including:

Bone cysts or pseudocysts

Despite their names – simple bone cyst and aneurysmal bone cyst – these entities are no longer categorized as cysts. In the new WHO Classification they come under the heading of bone-related lesions. They more commonly affect the skeleton and only relatively rarely affect the jaws.

Simple (solitary) bone cyst (Fig. 26.8)

Defined as an intra-osseous pseudocyst, devoid of an epithelial lining and either empty or filled with serous or sanguineous fluid. The aetiology is unknown.

Typical radiographic features of tumours and tumour-like lesions

Odontogenic tumours

Ameloblastoma (Fig. 26.9)

An aggressive but non-metastasizing tumour originating from remnants of the odontogenic epithelium of the enamel organ or dental lamina. Four main types include:

Solid/multicystic ameloblastoma

• Age: Adults 30–60 years old.

• Frequency: Rare, but still the most common odontogenic tumour.

• Site: —80% posterior body/angle/ramus of mandible

• Size: Very variable depending on the age of the lesion, may become very large if neglected and cause gross facial asymmetry.

• Shape: —Multilocular, distinct septa dividing the lesion into compartments with large, apparently discrete areas centrally and with smaller areas on the periphery

• Outline: — Smooth and scalloped

• Radiodensity: Radiolucent with internal radiopaque septa.

• Effects: —Adjacent teeth displaced, loosened, often resorbed

Ameloblastic fibroma (Fig. 26.10)

A rare, benign, mixed odontogenic tumour originating from both the odontogenic epithelium and the connective tissue of the developing tooth germ. Histologically, it consists of odontogenic ectomesenchyme resembling dental papilla and epithelial strands and rests resembling dental lamina and enamel organ. Radiographically these tumours closely resemble ameloblastomas but develop in a younger age group.

Other epithelial odontogenic tumours

The other more important epithelial odontogenic tumours, with or without odontogenic ectomesenchyme, that can present as a radiolucency include:

However, as the name of a couple of them suggests, these lesions often develop internal calcifications and more typically present as lesions of variable radiopacity. They are therefore described in detail with appropriate examples in Chapter 27. A brief summary of each is outlined below.

Calcifying epithelial odontogenic tumour (CEOT), Pindborg tumour

This rare odontogenic tumour usually presents in the premolar/molar region of the mandible in 20–60-year-old adults. They can be either unilocular or multilocular, but tend to remain relatively small although they can cause expansion of surrounding cortical bone. They are often associated with an unerupted tooth particularly image. The outline of the lesion tends to be of variable definition and cortication but is frequently scalloped. They are often radiolucent in their early stages; then numerous scattered radiopacities usually become evident within the lesion, often most prominent around the crown of any associated unerupted tooth. This appearance is sometimes described as driven snow. Adjacent teeth can be either displaced and/or resorbed (see Fig. 27.8).

Ameloblastic fibro-odontoma

These rare, unilocular or multilocular odontogenic tumours closely resemble ameloblastic fibromas (see Fig. 26.10), and also affect children. However, they are often associated with an unerupted tooth and usually contain enamel or dentine, either as multiple small opacities or as a solid mass (see Fig. 27.9).

Odontogenic fibroma (Fig. 26.11)

Controversy still exists as to the concept and definition of the odontogenic fibroma as a distinct lesion. The WHO classify it separately and describe it as a rare neoplasm characterized histologically by varying amounts of inactive-looking odontogenic epithelium embedded in a mature fibrous stroma. Two sub-types are described, thought to develop from different parts of the tooth germ:

Typically lesions present as cyst-like well defined, unilocular radiolucencies with dense corticated margin. Rarely calcified material may develop internally and adjacent teeth may be displaced.

Odontogenic myxoma (Fig. 26.12)

This intra-osseous neoplasm is characterized histologically by stellate and spindle-shaped cells embedded in an abundant myxoid or mucoid extracellular matrix originating from the odontogenic connective tissue fibroblasts of the developing tooth germ.

• Age: Young adults – most diagnosed in the 2nd–4th decades.

• Frequency: Rare, but the third most common odontogenic tumour.

• Site: Posterior mandible or posterior maxilla.

• Size: Variable, but may become very large if untreated.

• Shape: —Multilocular (honeycomb or soap-bubble)

• Outline: — Smooth and often scalloped

• Radiodensity: Radiolucent with fine internal radiopaque septa or trabeculae often arranged at right angles to one another, producing an appearance sometimes described as resembling the strings of a tennis racket or the letters X and Y.

• Effects: —Adjacent teeth displaced and loosened, occasionally resorbed

Radiolucent non-odontogenic tumours

Intrinsic primary benign bone tumours

Central haemangioma (Fig. 26.13)

This is a rare, benign tumour that occasionally affects the jaws, particularly the mandible. It is usually regarded as a developmental malformation (hamartoma) of the blood vessels in the marrow spaces, rather than a true neoplasm.

Central haemangioma can present at any age but is usually discovered in adolescents. It can produce a very variable radiographic appearance. These variations are important because of the life-threatening nature of the lesion; they include:

Intrinsic primary malignant bone tumours

Osteosarcoma (Fig. 26.14)

Rare, rapidly destructive malignant tumour of bone. From a radiological viewpoint, there are three main types:

Extrinsic primary malignant tumours involving bone

Squamous cell carcinoma (Fig. 26.15)

Squamous cell carcinomas of the oral mucosa directly overlying bone, in their latter stages, often invade the underlying bone to produce a destructive radiolucency.

Secondary (metastatic) bone tumours (Fig. 26.16)

Carcinomas from the bronchus, breast, prostate, kidney and thyroid sometimes metastasize to the jaws and produce the typical destructive radiolucency of a malignant lesion (see Ch. 21).

Note: The radiographic appearance, while strongly indicating a destructive malignant lesion, does not enable the distinction between a primary or secondary tumour to be made.

Lymphoreticular tumours of bone (Fig. 26.17)

Multiple myeloma (Fig. 26.17)

Multifocal proliferation of the plasma cell series within the bone marrow, resulting in overproduction of immunoglobulins.

Others

These lymphoreticular tumours are rare and apart from the age group predilection (shown above), they present in a similar, relatively non-specific manner. Radiographically they usually present as expansile, destructive, poorly defined, radiolucent areas – suggestive of malignant disease.

Langerhans cell disease (histiocytosis X)

Langerhans cell disease is used as a broad grouping of three different clinical manifestations of the same disease. All three manifestations produce tumour-like lesions in bone, caused by proliferation of Langerhans cells and eosinophilic leucocytes:

• Solitary eosinophilic granuloma – localized to the skeleton, affecting adolescents and young adults (see Fig. 26.18)

• Multifocal eosinophilic granuloma (Hand–Schüller–Christian disease) – chronic and wide-spread, begins in childhood but may not be fully developed until early adulthood, 20–30 years

• Letterer–Siwe disease – acute or subacute and widespread, affecting children under three years old.

Radiographically, the bone lesions (in whatever parts of the skeleton are affected) are similar in all three diseases.

Note: Appearance not suggestive of malignant disease.

Typical radiographic features of bone-related lesions

Giant cell lesions

Central giant cell lesion (granuloma) (Fig. 26.19)

A relatively rare localized, benign but sometimes aggressive osteolytic proliferation consisting histiologically of fibrous tissue with haemorrhage and haemosiderin deposits, presence of osteoclast-like giant cells and reactive bone formation producing an expansile radiolucent lesion.

Based on their clinical and radiological effects on adjacent structures, central giant cell lesions are sometimes subdivided into two categories:

Brown tumours in hyperparathyroidism

The general radiological features of hyperparathyroidism are discussed in detail in Chapter 31. A few patients with this disease, in addition to the generalized decrease in bone density, also develop circumscribed, cyst-like radiolucencies. Histologically and radiologically these individual lesions (so-called brown tumours) are indistinguishable from central giant cell lesions (see earlier).

Cherubism (Fig. 26.20)

This rare disease of the jaws is inherited, usually as an autosomal dominant, but some cases appear spontaneously. Radiologically the lesions resemble closely other giant cell-containing lesions.

Aneurysmal bone cyst (Fig. 26.21)

As stated earlier, despite its name this rare lesion is categorized as a giant cell lesion and is defined as an expansile osteolytic lesion, often multilocular with histologically blood-filled spaces separated by fibrous septa containing osteoclast-type giant cells and reactive bone.

Bone-related lesions – osseous dysplasias

The 2005 WHO Classification, shown in Table 26.1, now categorizes fibro-cemento-osseous lesions as osseous dysplasias and includes four conditions:

They are all skeletal disorders in which bone is replaced by fibrous tissue which in turn is replaced by bone or mineralized tissue to a varying degree as the lesions age. Thus, in their early stages all the osseous dysplasias can present as cyst-like radiolucencies, although they are only sometimes seen clinically at this stage. It is more common to see them in their later stages when they present as mixed radiolucent/radiopaque lesions with varying degrees of opacity. Two radiolucent examples are shown here (Figs 26.22 and 26.23). The more radiopaque lesions are discussed and illustrated in Chapter 27.

Florid osseous dysplasia (Fig. 26.23)

• Age: Middle-aged adults (typically black women).

• Frequency: Rare.

• Site: Widespread, often in all four quadrants (dentulous and edentulous) but associated with the apices of the teeth if present.

• Size: Variable, but individual lesions up to 2 cm in diameter.

• Shape: — Multiple

• Outline: — Smooth but lobular

• Radiodensity: —Early stage – multiple radiolucencies

• Effects: —Adjacent teeth – not displaced, not resorbed, typically vital

Other bone-related lesions

The other bone-related lesions that could present as a radiolucency include:

Stafne’s bone cavity (Fig. 26.24)

A bone cavity or depression on the lingual aspect of the mandible near the lower border, frequently said to contain aberrant salivary gland tissue.

Footnote

In view of this large number of radiolucent conditions, an ordered, systematic approach when producing a differential diagnosis is essential. Although the old adage that common things are commonly seen applies aptly to radiology, clinicians always have to be prepared for the possibility that they may be dealing with one of the rare, and perhaps sinister conditions.

For revision purposes, Table 26.2 summarizes those lesions that present typically as unilocular or multilocular radiolucencies.

Table 26.2

Summary of the main unilocular and multilocular radiolucent lesions

Typically unilocular lesions Typically multilocular or pseudolocular lesions
Radicular (dental) cyst
Residual radicular cyst
Odontogenic keratocyst (keratocystic odontogenic tumour)AmeloblastomaAmeloblastic fibromaCalcifying epithelial odontogenic tumourOdontogenic myxomaHaemangiomaGiant cell lesions:— Central giant cell lesion— Brown tumour— Cherubism— Aneurysmal bone cyst
Dentigerous cyst
Lateral periodontal cyst
Nasopalatine duct cyst
Simple (solitary) bone cyst
Calcifying epithelial odontogenic tumour
Calcifying cystic odontogenic tumour
Adenomatoid odontogenic tumour
Odontogenic fibroma
Primary bone tumours
Haemangioma
Secondary (metastatic) tumours  
Multiple myeloma  
Eosinophilic granuloma  
Osseous dysplasias (early stages)  
Stafne’s bone cavity  

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