Other Diseases Affecting the Jaws










199
Atlas of Oral and Maxillofacial Radiology, First Edition. Bernard Koong.
© 2017 John Wiley & Sons Ltd. Published 2017 by John Wiley & Sons Ltd.
13.1 Central giant cell granuloma
(Figures13.1–13.5)
Synonyms: CGCG, giant cell reparative granuloma.
A non‐neoplastic lesion consisting of vascular tissue and
multinucleated giant cells. Generally considered to be reactive
or reparative in nature.
Most commonly occurs in the jaws, especially the mandible.
Most often presents in the second and third decades of life.
When seen in the older person, hyperparathyroidism should
be considered (brown tumour).
While not a tumour, it often presents with tumour‐like clinical
features.
Usually surgical removal. Recurrence is relatively rare.
Radiological features
Multidetector computed tomography (MDCT) is the pre-
ferred imaging modality. However, cone beam computed
tomography (CBCT) may be sufficient for some cases.
Magnetic resonance imaging (MRI) can be useful for fur-
ther characterisation, especially in differentiating from the
aneurysmal bone cyst.
Well‐defined multilocular expansile lesion. The septa are
typically extremely fine, which may be difficult to identify,
especially on 2D radiography and CBCT. These septa are
often more obvious in soft tissue windows (MDCT). In
the classical lesion, one or two of these septa can be seen
at right angles to the expanded jaw cortices. The appear-
ances of these septa are similar to those of the aneurysmal
bone cyst.
Expanded cortices often demonstrate a lobulated appearance.
The expanded cortices are often largely preserved, although
focal cortical effacements are not uncommon, especially with
maxillary lesions.
Sometimes, the margins may be poorly defined, especially in
the maxilla, resulting in a more aggressive appearance.
Can be unilocular, especially small lesions.
When sufficiently large, tooth displacement and root resorp-
tion are often seen.
Differential diagnosis
Key radiological differences
Other lesions
which may appear
to be multilocular,
including:
Ameloblastoma Thick and curved internal septa.
Odontogenic
myxoma
Presence of one or a few straight septa is
a feature. Typically demonstrates mild
expansion for size.
Aneurysmal bone
cyst (ABC)
Fine internal septa can appear very
similar to the CGCG. However, the ABC
is typically extremely expansile. MDCT
soft tissue windows may demonstrate
focal regions of fluid attenuation. The
characteristic fluid–fluid level is a feature
of the ABC which is well demonstrated
on MRI, and occasionally appreciated on
MDCT (narrow width soft tissue
window).
Brown tumour Can be identical radiologically and
histologically. The brown tumour is related
to hyperparathyroidism and theassociated
osteopenic changes may beseen.
CHAPTER 13
Other Diseases Affecting theJaws
Bernard Koong and Tom Huang

200 Atlas of Oral and Maxillofacial Radiology
Cysts The unilocular CGCG can resemble a
cyst, especially the simple bone cyst in
the younger patient. However, the simple
bone cyst typically does not displace or
resorb tooth roots.
Ossifying fibroma When this lesion demonstrates internal
septa, they tend to be larger.
Keratocystic
odontogenic
tumour
Internal septa are uncommon and this
lesion demonstrates only mild expansion
for its size.
Vas cu lar
malformation/
haemangioma
Often demonstrates serpiginous appear-
ances. Can appear to be multilocular on
2D radiography. Prebiopsy evaluation
with MRI should be considered if this is
suspected.
Cherubism Can be radiologically identical but
cherubism is usually multifocal, occurring
posteriorly.
Expansile multilocular
lesion with displacement
of 47 and 48. Note the
appearance of several
faint septa
Figure13.1 Central giant cell granuloma of the right posterior mandible: cropped panoramic radiograph.

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199Atlas of Oral and Maxillofacial Radiology, First Edition. Bernard Koong. © 2017 John Wiley & Sons Ltd. Published 2017 by John Wiley & Sons Ltd.13.1 Central giant cell granuloma (Figures13.1–13.5)• Synonyms: CGCG, giant cell reparative granuloma.• A non‐neoplastic lesion consisting of vascular tissue and multinucleated giant cells. Generally considered to be reactive or reparative in nature.• Most commonly occurs in the jaws, especially the mandible.• Most often presents in the second and third decades of life. When seen in the older person, hyperparathyroidism should be considered (brown tumour).• While not a tumour, it often presents with tumour‐like clinical features.• Usually surgical removal. Recurrence is relatively rare.Radiological features• Multidetector computed tomography (MDCT) is the pre-ferred imaging modality. However, cone beam computed tomography (CBCT) may be sufficient for some cases. Magnetic resonance imaging (MRI) can be useful for fur-ther characterisation, especially in differentiating from the aneurysmal bone cyst.• Well‐defined multilocular expansile lesion. The septa are typically extremely fine, which may be difficult to identify, especially on 2D radiography and CBCT. These septa are often more obvious in soft tissue windows (MDCT). In the classical lesion, one or two of these septa can be seen at right angles to the expanded jaw cortices. The appear-ances of these septa are similar to those of the aneurysmal bone cyst.• Expanded cortices often demonstrate a lobulated appearance. The expanded cortices are often largely preserved, although focal cortical effacements are not uncommon, especially with maxillary lesions.• Sometimes, the margins may be poorly defined, especially in the maxilla, resulting in a more aggressive appearance.• Can be unilocular, especially small lesions.• When sufficiently large, tooth displacement and root resorp-tion are often seen.Differential diagnosisKey radiological differencesOther lesions which may appear to be multilocular, including:Ameloblastoma Thick and curved internal septa.Odontogenic myxomaPresence of one or a few straight septa is a feature. Typically demonstrates mild expansion for size.Aneurysmal bone cyst (ABC)Fine internal septa can appear very similar to the CGCG. However, the ABC is typically extremely expansile. MDCT soft tissue windows may demonstrate focal regions of fluid attenuation. The characteristic fluid–fluid level is a feature of the ABC which is well demonstrated on MRI, and occasionally appreciated on MDCT (narrow width soft tissue window).Brown tumour Can be identical radiologically and histologically. The brown tumour is related to hyperparathyroidism and theassociated osteopenic changes may beseen.CHAPTER 13Other Diseases Affecting theJawsBernard Koong and Tom Huang 200 Atlas of Oral and Maxillofacial RadiologyCysts The unilocular CGCG can resemble a cyst, especially the simple bone cyst in the younger patient. However, the simple bone cyst typically does not displace or resorb tooth roots.Ossifying fibroma When this lesion demonstrates internal septa, they tend to be larger.Keratocystic odontogenic tumourInternal septa are uncommon and this lesion demonstrates only mild expansion for its size.Vas cu lar malformation/haemangiomaOften demonstrates serpiginous appear-ances. Can appear to be multilocular on 2D radiography. Prebiopsy evaluation with MRI should be considered if this is suspected.Cherubism Can be radiologically identical but cherubism is usually multifocal, occurring posteriorly.Expansile multilocular lesion with displacement of 47 and 48. Note the appearance of several faint septaFigure13.1 Central giant cell granuloma of the right posterior mandible: cropped panoramic radiograph. Other Diseases Affecting theJaws 201Expansile multilocular lesion with fine internal septaLobulated expansion with focal effacement of the corticesInternal soft tissue attenuation(a) (b)(c)Figure13.2 Central giant cell granuloma of the right posterior mandible: axial and corrected sagittal bone (a,c) and axial soft tissue (b) MDCT images.(a) (b)Expansile multilocular lesionsin the anterior maxilla withfine internal septaEffacement of theanterior borderof the displaced incisive canalLobulated expansion with small focal palatal cortical effacements. Note a septum demonstrating a right-angled relationship with this expanded borderFigure13.3 Central giant cell granuloma of the anterior maxilla: axial (a) and sagittal (b) MDCT images. 202 Atlas of Oral and Maxillofacial RadiologyExpansile unilocular lesion with preserved cortex. No internal septa demonstrated. Note the slightly lobulated morphology(a)(b)(c)Figure13.4 Central giant cell granuloma of the left coronoid process: axial (a), coronal (b) and corrected sagittal (c) MDCT images.Expansile lesions with focal thinning and effacement of the lingual cortexFine internal septa(a) (b)(c)Figure13.5 Brown tumours within the mandibular body, related to hyperparathyroidism: axial CBCT images (a–c). Other Diseases Affecting theJaws 20313.2 Cherubism (Figure13.6)• Synonyms: familial multilocular cystic disease of the jaw, familial fibrous jaw dysplasia.• Autosomal dominant familial disorder.• Occurs in the mandible more than in the maxilla.• Usually presents as a bilateral mandibular enlargement. Expansion related to large maxillary lesions, when present, may cause retraction of the lower eyelids and expose the sclera, resulting in the classically described clinical appearance of upward gaze.• The lesion usually develops in early childhood and progresses until puberty, when it may regress. After the lesion becomes static, surgery may be required for cosmetic or orthodontic reasons.Radiological features• Bilateral, well‐defined, expansile, multilocular lesions, usually centred in the posterior mandible and/or maxilla. Occasionally seen anteriorly.• Internal septa are usually fine and wispy. There may be septa which are thicker and more obvious in appearance.• Can cause tooth displacement and failure of eruption.Differential diagnosisKey radiological differencesCentral giant cell granulomaSimilar radiographic appearance but usually not bilateral.Aneurysmal bone cystSimilar radiographic appearance but usually not bilateral.Expansile multilocular lesions with variably dense internal septa. There is effacement of the jaw cortices. Note the displacement and associated failure of eruption of the teethSoft tissue attenuation between the septa(a)(b)(c)(d)(e)Figure13.6 Cherubism: lesions within the mandible (anterior and posterior) and posterior maxilla bilaterally: panoramic radiograph (a) with axial bone (b), coronal bone (c), axial soft tissue (d) and coronal soft tissue (e) MDCT images. 204 Atlas of Oral and Maxillofacial Radiology13.3 Aneurysmal bone cyst (Figure13.7)• Synonym: ABC.• A benign bone lesion composed of non‐endothelialised blood‐filled spaces separated by septa which consists of fibro-blasts, giant cells and reactive bone/osteoid.• Uncertain aetiology but generally not considered to be a true tumour or cyst. Some consider the ABC to be a reactive lesion.• May be secondary to an underlying lesion, including fibrous dysplasia, chondroblastoma and giant cell tumour.• Typically seen in the long bones.• Usually surgical removal. Recurrence is relatively high. May require resection and/or chemical treatment.Radiological features• MDCT is the first imaging modality of choice. However, fea-tures on CBCT and 2D radiography may be contributory. MRI can be particularly useful, demonstrating the characteristic fluid–fluid levels.• Well‐defined multilocular expansile lesion. Small lesions can be unilocular.• The internal septa are typically extremely fine, which may be difficult to identify, especially on 2D radiography and CBCT. These are often more obvious in MDCT soft tissue windows. These septa are similar to those of CGCG. There may also be septa that are at right angles to the expanded jaw cortices, also seen in CGCG.• MDCT soft tissue windows may demonstrate focal regions of fluid attenuation. Occasionally, fluid–fluid levels can be appreciated when a narrow window width is employed (much better appreciated on MRI).• Expansion is a feature when large, more than CGCG.• When sufficiently large, there may be associated tooth dis-placement and root resorption.• MRI: when present, fluid–fluid levels are well demonstrated. However, it should be noted that fluid–fluid levels may be seen in other lesions, including the giant cell tumour and rarely the simple bone cyst. Gadolinium‐enhanced septa may be seen.Differential diagnosisKey radiological differencesOther lesions which may appear to be multilocular, including:Ameloblastoma Thick and curved internal septa.Odontogenic myxoma Presence of one or a few straight septa is a feature. Typically demon-strates mild expansion forsize.Giant cell granuloma Fine internal septa can appear very similar to the ABC. However, the ABC is typically extremely expansile.Brown tumour Essentially radiologically and histologically identical to the CGCG. The brown tumour is related to hyperparathyroidism and associated osteopenic changesmay be seen.Cysts The small unilocular ABC may resemble a cyst.Ossifying fibroma When this lesion demonstrates the appearance of internal septa, they tend to be larger than those seen in the ABC.Keratocystic odontogenic tumourInternal septa are uncommon and this lesion demonstrates only mild expansion for its size.Vascular malformation/haemangiomaOften demonstrates serpiginous appearances. Can appear multilocu-lar on 2D radiography. Prebiopsy evaluation with MRI should be considered if this is suspected.Cherubism Can be radiologically similar but cherubism is usually multifocal, occurring posteriorly. Other Diseases Affecting theJaws 20513.4 Langerhans cell histiocytosis (Figures13.8–13.12; see also Figure20.16)• Synonyms: LCH, histiocytosis X.• A rare disease involving abnormal clonal proliferation of Langerhans cells (dendritic cells) which clinically manifests according to a number of subtypes which range from a solitary bone lesion to multisystem disease. The course also ranges from spontaneous remissions to aggressive acute disseminated disease.• Historical subdivision of overlapping clinical variants are controversial, with variation in definitions among clinicians and authors: ◦Eosinophilic granuloma. ■ Some consider this term to refer to the solitary lesion. Others consider it to refer to lesions confined to one organ. Most commonly seen in bone, where it may be monostotic or polyostotic. ■ Most commonly seen in children and younger adults. ■ Prognosis is good when there are only skeletal lesions, especially when solitary. ◦Hand–Schüller–Christian disease. ■ Multiple disseminated lesions. Some consider the term to refer to patients with several lesions within one organ and others accept involvement of multiple organs. Still others have used this term to refer to a triad of multiple bone lesions, diabetes insipidus and exophthalmos. ■ Most commonly seen in children. ◦Letterer–Siwe disease. ■ Disseminated, multisystem, rapidly progressive disease, often considered as the malignant form. ■ Most commonly in children under 2 years old. ■ Poor prognosis.• More recent classifications involve the extent of disease and also subdivision of non‐malignant and malignant forms.• In the jaws, the lesions may be solitary or multiple.• Treatment includes surgical excision, radiotherapy and chem-otherapy, depending on the extent of disease.Radiological features (jaw lesions)• MDCT and MRI are the imaging modalities of choice. However, features on CBCT and 2D radiography may be con-tributory. Both single‐photon emission computed tomogra-phy (SPECT) and positron emission tomography (PET) with or without CT may assist in the staging of the disease but there is a significant false‐negative rate in bone and gallium scanning. PET imaging findings may also be misleading given the variability in the presentation of the disease.• Lytic destructive lesion(s). However, the borders range from relatively well defined, including ‘punched out’ borders, to ill‐defined aggressive appearances.• When involving the alveolar processes, they tend to be multi-focal, most commonly involving the posterior mandible. A useful feature is that these lesions are usually centred upon the middle third of the tooth roots. These lesions destroy bone around teeth and may eventually result in the ‘floating teeth’ appearance.• Alveolar lesions may be secondarily infected, with associated radiological changes including adjacent reactive sclerosis.• Elsewhere in the jaws, it destroys jaw cortices and is usually associated with soft tissue mass. The laminar periosteal response is a feature not usually seen with alveolar lesions.• MRI: low to intermediate T1 signal, intermediate to high T2 and short T1 inversion recovery (STIR) signal. Enhancement is demonstrated with intravenous gadolinium.Substantially expansile multilocular lesion with the appearance of faint internal septa Fluid–fluid level Enhancement of septa(a) (b)Figure13.7 Aneurysmal bone cyst of the left mandible: cropped panoramic radiograph (a) and postcontrast axial soft tissue MDCT image (b). (Courtesy of Professor Paul Monsour, School of Dentistry, The University of Queensland, Australia.) 206 Atlas of Oral and Maxillofacial RadiologyDifferential diagnosisKey radiological differencesPlaque‐related inflammatory peri-odontal bone lossPlaque‐related inflammatory periodontal bone loss begins at the alveolar crest and extends apically. LCH lesions involving the alveolar processes are usually centred on the midroot surface, where preservation of the alveolar crests may be seen at the leading edges of the lesion(s).Malignant lesions, including lymphoma, leukaemia, Ewing sarcoma and metastatic disease. Bony invasion of adjacent soft tissue malignancies (e.g. squamous cell carci-noma) should also be consideredLCH alveolar lesions are usually centred upon the midroot surfaces. Also, these lesions tend to be more well defined in appearance than malignant lesions. When it is seen elsewhere in the jaws, there is usually a laminar periosteal response.The morphology of the mesial extension of this periradicular lucent defect is not typical of plaque-related periodontal bone loss.Of note is the preservation of the bone at the furcationFigure13.8 Langerhans cell histiocytosis of the left mandibular alveolar process: cropped panoramic radiograph.Lytic lesion in the right mandibular angle with inferior lamina periostealreactionFigure13.9 Langerhans cell histiocytosis of the left posterior mandible: oblique mandible radiograph. Lucent lesion with cortical destruction and laminaperiosteal reactionSoft tissue mass with heterogeneous enhancementMixed T2 signal soft tissue massLamina periosteal responseRelatively well-defined lucent lesion with irregular borders(a) (c)(b) (d)Figure13.11 Langerhans cell histiocytosis of the right body of the mandible: coronal postcontrast soft tissue (a) and axial bone (b) MDCT images, cropped panoramic radiograph (c) and coronal T2 MRI image (d).Lucent lesion withcortical destruction andlamina periosteal reactionSoft tissue mass lesion(a) (b) (c)Figure13.10 Langerhans cell histiocytosis–solitary lesion at the right‐angle of the mandible: axial bone (a), coronal bone (b) and coronal soft tissue (c) MDCT images. 208 Atlas of Oral and Maxillofacial Radiology(a) (c)(b)(d)Lytic lesionSoft tissue mass withheterogeneousenhancement Lucent lesion with corticaldestruction. Notedestruction of the molarfollicular cortexFigure13.12 Langerhans cell histiocytosis of the left mandible and skull: posteroanterior radiograph (a) with axial bone (b,d) and coronal postcontrast soft tissue (c) MDCT images.13.5 Paget disease ofbone (Figure13.13; seealso Figure20.18)• Synonym: osteitis deformans.• Chronic disorder of the bone characterised by aberrant bone remodelling and formation of bone that is structurally abnor-mal, with expansion and deformity.• Usually presents in the fourth decade of life or beyond.• While most are symptom free, some present with skeletal deformities and pain and ill‐fitting dentures. May also present with associated cranial neuropathies.• Bones most frequently involved: skull, sacrum, spine, pelvis and lower extremities.• It is rarely found in the jaws, more often in the maxilla. Usually bilateral.• Raised serum alkaline phosphatase levels.• Rarely, other tumours including the osteosarcoma may arise.Radiological features (jaw lesions)• In the incipient (lytic) phase, lucent appearances are noted.• In the intermediate phase (lysis and sclerosis), it is heterogeneous and may demonstrate a ‘cotton wool’ appearance.• A later sclerotic stage follows. The trabecular bone may dem-onstrate homogeneous regions and other regions of coarse trabeculae where a linear pattern may be seen.• Expansion is also a feature.• There is usually displacement of teeth and alteration of the lamina dura. Hypercementosis may be seen. Other Diseases Affecting theJaws 209Differential diagnosisKey radiological differencesFibrous dysplasia Fibrous dysplasia occurs in a younger age group. Ground‐glass appearance is seen in the classical fibrous dysplasia. Paget disease is usually bilateral.Chronic osteomyelitis A periosteal response is almost always present in chronic osteomyelitis.Metastatic disease In the lytic phase, it may be difficult todifferentiate. If there is sclerosis without bone expansion, then prostate or breast metastasis is favoured.Osteopetrosis Presents in the young. Diffused dense sclerosis.Expanded body of themandible with thinningand alteration of thecortical architectureRegions demonstrating a linear patternA homogeneouslysclerotic regionAltered architecture ofthe lamina dura of themandibular teeth(a)(b)Figure13.13 Paget disease: axial MDCT images (a,b).

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