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Pe d i a t r i c I m a g i n g • R ev i ew

Plunk et al.
Benign Pediatric Mandible Disorders

Pediatric Imaging
Review

Focal Benign Disorders of


the Pediatric Mandible With
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Radiologic-Histopathologic
Correlation: Mandibular
Development and Lucent Lesions
Matthew R. Plunk1,2 OBJECTIVE. Lucent lesions of the pediatric mandible may present variably. Cysts, neo-
Dolphine Oda3 plasms, and developmental and inflammatory conditions have a host of possible causes. There
Shawn E. Parnell1,4 is also substantial overlap in the imaging appearance of cysts and that of benign but locally
Jason N. Wright 1,4 aggressive tumors that need to undergo resection.
Bonnie L. Cole 3,5 CONCLUSION. The purpose of this article is to present common and uncommon lucent
lesions of the mandible in children, with an emphasis on benign abnormalities. Discussions of
Ramesh S. Iyer 1,4
imaging and histopathologic features are provided.
Plunk MR, Oda D, Parnell SE, Wright JN, Cole BL,
bnormalities of the mandible in

A
Iyer RS Mandibular and Dental Development
children are frequently identified and Terminology
and treated exclusively by dentists A basic understanding of the develop-
and oral surgeons with in-office ment of the jaw and dentition will be useful
imaging (e.g., panoramic radiography) and for framing future discussions [3, 4]. During
procedures. Radiologists may also be called the fourth week of gestation, the embryon-
on to characterize a mandibular abnormality, ic maxilla and mandible arise from enlarge-
either when a patient presents with symptoms ment and fusion of paired bilateral promi-
to a health care facility or when the abnormal- nences derived from neural crest cells around
ity is incidentally found on an imaging study the primitive mouth, known as the stomode-
performed for another purpose. um. The skeleton of the mandible is derived
Keywords: lucent lesions, mandibular tumors, nonlucent The mandible represents an intersection of using Meckel cartilage of the first pharynge-
lesions, pediatric mandible, radiologic-histopathologic two separate realms of disease: one associated al arch as a template for membranous ossifi-
correlation with tooth development and the other a prima- cation. Interestingly, this cartilage does not
ry bone process. Odontogenic neoplasms, in contribute to the final mandible, but portions
DOI:10.2214/AJR.16.16587
particular, can be troublesome because of sim- are retained as middle ear structures, includ-
Received April 21, 2016; accepted after revision ilarities in nomenclature and because several ing the malleus and incus.
July 18, 2016. entities have been reclassified, renamed, and The teeth are derived from a combination
1
discovered with recent advances in molecular of ectoderm and ectomesenchyme of the head,
Department of Radiology, University of Washington
School of Medicine, Seattle, WA.
biology and immunohistochemistry [1, 2]. which is itself derived from neural crest cells.
This article reviews the radiographic and Tooth development is a complex process that is
2
Present address: Department of Radiology, T­ exas  pathologic features of acquired odontogenic beyond the scope of this review. Simplified, the
Children’s Hospital, Houston, TX. and nonodontogenic abnormalities, with the overlying ectoderm, or dental lamina, invag-
3 goal of helping generate a reasonable differ- inates into the underlying ectomesenchyme.
Department of Anatomic Pathology, University of
Washington School of Medicine, Seattle, WA. ential diagnosis. There is an emphasis on be- This invagination induces cellular prolifera-
nign disorders, particularly cysts and nonma- tion in the ectomesenchyme, which subse-
4
Department of Radiology, Seattle Children’s Hospital, lignant tumors, which may be distinguished quently forms the dental papilla. These devel-
4800 Sand Point Way NE, MA.7.220, Seattle, WA 98105. by particular patient age ranges, their rela- oping ectomesenchymal cells are contained in
Address correspondence to R. S. Iyer (riyer@uw.edu).
tionship to teeth, and their location within a sac known as the dental follicle. Eventually,
5
Department of Laboratories, Seattle Children’s Hospital, the mandible. A summary of the lucent le- the overlying dental lamina forms the amelo-
Seattle, WA. sions presented in this article appears in Ta- blasts (which produce the outer tooth enamel),
ble 1. Malignant and malignantlike abnor- the dental papilla forms the dental pulp and
AJR 2017; 208:1–13 malities producing lucency in the pediatric the odontoblasts (which produce dentin), and
0361–803X/17/2081–1
mandible, such as osteosarcoma and Langer- the follicle forms the cementum as well as the
hans cell histiocytosis, are beyond the scope periodontal ligament (which anchors the tooth
© American Roentgen Ray Society of this article. to the underlying alveolar bone).

AJR:208, January 2017 1


Plunk et al.

TABLE 1: Summary of Focal Lucent Lesions


Name Imaging Features Presentation and Associations
Dentigerous cyst Expansile well-circumscribed radiolucency Most common pediatric mandible lesion after dental caries;
most common odontogenic cyst
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Often associated with crown of unerupted tooth May be multiple in cleidocranial dysplasia or Maroteaux-
Lamy syndrome
Periapical cyst Round or ovoid lucency associated with root of the tooth Associated with dental caries
May be multiple; may be large if untreated If painful, may be periapical granuloma or abscess
Keratocystic odontogenic tumor Unilocular, expansile, usually solitary lucency growing Previously known as odontogenic keratocyst
along axis of mandible with mass effect on adjacent teeth
If MRI performed, no solid enhancing components and may Jaw swelling and pain are common because of expansion
be intrinsically T1 hyperintense
Multiple lesions in basal cell nevus syndrome
Ameloblastoma Variable appearance given tissue composition heterogeneity Overall the most common primary mandibular tumor in
children
Uni- or multilocular expansile lucent lesion of posterior Unicystic tumors are most common in children, though solid
mandible, often associated with impacted tooth (unicystic tumors are overall most common subtype
subtype)
Aggressive features such as tooth resorption may be
present but can also mimic benign lesion
Avidly enhancing solid components may be present on
cross-sectional imaging
Odontogenic myxoma Imaging appearance similar to ameloblastoma Uncommon tumor often distinguished from ameloblastoma
only by pathologic analysis
Benign but locally aggressive lesion
Adenomatoid odontogenic tumor More common in anterior maxilla and mandible, a Rare tumor
distinguishing feature
Unilocular lucency sometimes associated with unerupted Two-thirds tumor
tooth, which can mimic a dentigerous cyst
Bone cyst (simple and aneurysmal) Simple bone cysts have unilocular lucency; may displace Simple bone cysts often asymptomatic; pathologic fracture
teeth but without cortical expansion uncommon in mandible
Aneurysmal bone cysts are expansile and multilocular, with Aneurysmal bone cyst may have indolent or rapid growth
fluid-fluid levels
Central giant cell granuloma Unilocular lucency located in the anterior mandible, often Histologically indistinguishable from cherubism or brown
parasymphyseal tumors of hyperparathyroidism
May have aggressive or nonaggressive radiographic
appearance

Humans have 20 primary teeth and 32 Lucent Lesions formulated. Lucent lesions of the pediatric
permanent teeth that replace them. The per- Radiolucent lesions are frequent in the mandible incorporate both odontogenic and
manent teeth arise from a secondary dental mandible. The lucent appearance of these le- nonodontogenic lesions and both cystic and
lamina that is located lingual and deep to the sions may be a consequence of cystic expan- neoplastic processes.
primary lamina, with the exception of the sion or erosion of adjacent bone. Making this
first through third adult molars, which arise distinction is difficult with radiography, and Odontogenic Cysts
from the primary lamina. All the developing cross-sectional imaging such as CT or MRI Dentigerous Cyst
tooth buds are typically present at birth ex- may be helpful. For example, at many centers, Dentigerous cyst, or follicular cyst, is not
cept those for the second and third molars. the use of panoramic radiography for imaging only the most common pediatric odontogen-
The crown is the enamel-covered superior lucent lesions of the jaws has been replaced ic cyst, it is the most frequently encountered
portion of the tooth that is visible after a tooth by cone-beam CT for greater anatomic detail. lesion of the mandible in children after den-
has erupted. The root is the inferior portion When characterizing a lucent lesion of tal caries [5, 6]. It results from expansion
that is covered with cementum and is not usu- the mandible, it is important to recognize and fluid accumulation within remnants of
ally visible. The term “pericoronal” is used to that the imaging appearances of these enti- the dental lamina and usually presents in the
refer to a finding associated with the crown of ties frequently overlap and further character- second decade of life [7]. Dentigerous cysts
the tooth, whereas the terms “periradicular” ization may not be possible. However, a rea- are typically asymptomatic unless they are
and “periapical” refer to the apex of a root. sonable differential diagnosis can often be large with associated mass effects on the

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Benign Pediatric Mandible Disorders

teeth, are infected, or are complicated by the periodontal ligament, producing an epi- genic tumor tends to grow along the axis of
pathologic fracture. thelium-lined cavity filled with sterile fluid the mandible with anterior-posterior hollow-
These cysts appear radiographically as uni- [19]. Although radiographs are typically per- ing of the bone and relatively less transverse
locular well-circumscribed radiolucencies formed to aid in diagnosis, it can be difficult expansion (Figs. 4B and 4C). Keratocystic
that classically are associated with the crown to differentiate periapical cysts from periapi- odontogenic tumor can be associated with the
of an unerupted tooth (Figs. 1 and 2). They cal granulomas or abscesses on the basis of crown of an unerupted tooth but not as com-
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are frequently expansile and can resorb or dis- radiographic appearance, although lesions monly as dentigerous cysts [23]. CT will often
place the roots of adjacent teeth, but the over- greater than 1 cm in diameter are more likely better show the thinning of the surrounding
lying cortex is preserved. The third molar is to be periapical cysts [20, 21]. mandibular cortex. If MRI is performed, ker-
the most common location [7]. Although den- Periapical cysts usually present as round atocystic odontogenic tumor has been shown
tigerous cysts typically have a nonaggressive or ovoid lucencies associated with the root of to show intrinsic high T1 signal and hetero-
appearance, more aggressive or malignant the tooth (Fig. 3) but occasionally can be lo- geneous T2 signal, attributed to high keratin
conditions can coexist within or mimic their cated lateral to a tooth, where they are called and cholesterol contents in the lumen, and rim
appearance, and histologic analysis is recom- “lateral radicular cysts.” They may be mul- enhancement. The lack of solid enhancing
mended for suspected cases [8, 9]. tiple in the case of multiple dental caries. components can help distinguish a keratocys-
Histologically, dentigerous cysts are sim- They are typically small but may exceed 1 tic odontogenic tumor from ameloblastoma
ple epithelium-lined unilocular cystic lesions cm in diameter if left untreated. At this size, [28, 29]. The dense contents of a keratocys-
surrounded by a connective tissue wall (Fig. they can result in resorption of adjacent tooth tic odontogenic tumor have been shown to re-
1C). The cysts are usually lined by nonkera- roots, as well as possible pathologic fracture strict diffusion, which may further distinguish
tinizing squamous epithelium, but occasion- and rupture of the cyst contents into adjacent them from other cystic lesions [30].
ally columnar or cuboidal epithelium with soft tissues [22]. Keratocystic odontogenic tumors, as the
mucous-producing cells is a prominent com- The histologic appearance of periapical name suggests, have a cystic appearance un-
ponent of the cyst lining. The connective tis- cysts is similar to that of dentigerous cysts, der the microscope, with cysts lined by ke-
sue wall ranges from loose and myxoid with and clinical and radiographic correlations ratinized squamous epithelium (Fig. 4D).
very little inflammation, in children, to fi- are often needed to discriminate between The epithelial lining is typically uniform in
brotic and inflamed, in adults. these two lesions. Under the microscope, thickness, has a prominent parakeratin cell
Multiple or bilateral dentigerous cysts are nonkeratinizing squamous epithelium is seen layer, and lacks rete pegs. The basal layer is
rare in isolation and typically are associated to form the cystic lining, which is surround- usually palisaded, with hyperchromatic nu-
with such syndromes as cleidocranial dyspla- ed by a wall of connective tissue in which clei present in the cells. The cystic wall con-
sia and Maroteaux-Lamy syndrome [10, 11]. inflammation and hemorrhage are prominent nective tissue shows a cellular to fibrotic ap-
In general, dentigerous cysts associated with (Fig. 3C). Foci of foreign body giant cell re- pearance, whereas in the cystic lumen, layers
a supernumerary tooth are uncommon [12]. action and cholesterol clefts may be present. of sloughed keratin fibers may be present.
Special note should be made of the erup- Keratocystic odontogenic tumors are usu-
tion cyst, which had previously been consid- Lucent Odontogenic Neoplasms ally solitary. However, if multiple tumors are
ered a subtype of dentigerous cyst, because it Keratocystic Odontogenic Tumor detected, the possible diagnosis of nevoid bas-
shares the same histologic appearance. Erup- Because of their more aggressive nature al cell carcinoma (Gorlin-Goltz syndrome)
tion cysts are apparent clinically as mucosal compared with other odontogenic cysts [23, should be entertained, because multiple kera-
swelling and discoloration overlying a soon- 24], lesions formed by neoplastic prolifera- tocystic odontogenic tumors may be the first
to-erupt tooth, and they are managed expec- tion of remnants of the dental lamina, pre- recognized manifestation of this syndrome
tantly [13, 14]. If a radiograph is obtained for viously known as odontogenic keratocysts [31]. Up to 75% of patients with nevoid bas-
characterization, no osseous abnormalities (OKCs), were reclassified in 2005 by the al cell carcinoma syndrome may have multi-
will be present. World Health Organization as tumors [2]. ple keratocystic odontogenic tumors, so when
These lesions are now identified as kerato- they are present, a search should be made for
Periapical Cyst cystic odontogenic tumors. At the same time, other manifestations of Gorlin-Goltz syn-
Periapical cysts, also known as radicular the orthokeratinized subtype of OKC was re- drome, including dural calcifications, axial
cysts, are the most common dental lesions in named “orthokeratinized odontogenic cyst” skeletal abnormalities, and medulloblastoma.
adults [15]. Periapical cysts are most com- [25, 26], retaining its classification as a non- A solitary keratocystic odontogenic tumor
mon in the fifth decade of life, and only about tumor cyst. The peak incidence of keratocys- does not have malignant potential but may be
10% of all periapical cysts in one series oc- tic odontogenic tumors occurs in the third locally aggressive, grow rapidly, and perfo-
curred in patients younger than 17 years [16– decade of life, but these tumors may also be rate bone. It also carries a higher risk of local
18]. Although they also occur commonly in seen in children and adolescents. recurrence after simple enucleation than that
children, they occur less frequently in the On radiography, the keratocystic odonto- seen with other odontogenic cysts [32].
pediatric population than in older patients. genic tumor will appear as a unilocular radio-
These acquired odontogenic cysts are the re- lucency with a scalloped margin, usually lo- Ameloblastoma
sult of cytokine production by inflamed or cated in the posterior mandible [27] (Fig. 4A). Ameloblastoma is overall the most com-
necrotic pulp in the involved tooth after den- There will often be mass effect on the adja- mon primary mandibular tumor in children,
tal caries or trauma. The cytokines induce cent teeth with splaying, displacement, or re- and it may have a variety of appearances de-
proliferation of residual epithelial cells in sorption of their roots. Keratocystic odonto- pending on its tissue composition. Amelo-

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Plunk et al.

blastomas are divided into four categories: lar connective tissue (Fig. 6B). Inflammation the maxilla, they usually invade the maxil-
unicystic, solid, desmoplastic, and peripheral is usually absent. The epithelial islands are lary sinuses and may rarely cross the midline
(extraosseous). All types of ameloblastomas lined by a single layer of palisaded cuboidal to involve the opposite sinus. Most such tu-
are benign, but locally aggressive, neoplasms and columnar cells with reversed polariza- mors are expansile multilocular lesions with
with a high risk of local recurrence. tion and hyperchromatic nuclei. Because the internal trabeculations; they can displace
Unicystic ameloblastomas are the most recurrence rate of these lesions ranges from and resorb teeth [46]. Microscopic examina-
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common type of mandibular tumor in chil- 50% to 90%, the treatment of choice for the tion shows the lesions to be made up of loose
dren. They are associated with the crown of solid ameloblastoma is en bloc resection with and delicate fibrous connective tissue. The
an impacted tooth in about 90% of cases. The adequate clean margins. lesions are typically gelatinous, making cu-
radiographic appearance in these cases is sim- MRI has been investigated in character- rettage alone difficult. Surgical excision with
ilar to that of a dentigerous cyst, possibly ac- izing ameloblastoma and distinguishing it adequate margins is the preferred interven-
companied by a small amount of enhancing from other similar-appearing cystic man- tion to prevent local recurrence.
soft tissue (Figs. 5 and 6). The other 10% of dibular lesions, though use of this modality
unicystic ameloblastomas are unilocular ra- is not currently widespread. The cystic por- Adenomatoid Odontogenic Tumor
diolucencies usually present between teeth. tions of ameloblastoma typically show low- Often detected as an incidental finding on
Around 10–20% of all ameloblastomas occur to-intermediate T1-weighted signal and high dental radiographs, adenomatoid odontogen-
in patients 14–20 years old, with rare cases in T2-weighted and STIR signal [37]. Gado- ic tumor is a rare benign slow-growing neo-
children as young as 2–3 years old [33–36]. linium-enhanced sequences can be helpful plasm of odontogenic epithelium that is often
Three histologic subtypes of unicystic am- in identifying ameloblastoma: Although the initially misdiagnosed as a more common
eloblastomas have been described: luminal, cystic portions are typically nonenhancing, dentigerous cyst, which has a similar radio-
intraluminal unicystic (plexiform), and mural an enhancing solid mural component may logic appearance. Adenomatoid odontogenic
unicystic (Fig. 5C). Unicystic ameloblastomas be identified even in predominantly cys- tumor is sometimes referred to as the two-
behave less aggressively than do multilocular tic lesions, which favors ameloblastoma as thirds tumor, because two-thirds of cases
solid ameloblastomas. Curettage is therefore the diagnosis [38, 39]. Ameloblastoma has occur in young female patients aged 10–19
the preferred treatment of the luminal and been shown to enhance more rapidly and years, two-thirds occur in the maxilla, two-
intraluminal histologic subtypes of unicys- avidly than keratocystic odontogenic tumor thirds are located anteriorly in the jaw, and
tic ameloblastomas, whereas en bloc removal in adults [40, 41]. DWI may also have some two-thirds occur in association with an un-
or resection is the treatment of choice for the utility in ameloblastoma, because the non- erupted tooth [47]. One unique feature that
mural subtype. Recurrence rates range from enhancing portions of ameloblastoma have may suggest the diagnosis is that adenoma-
6% to 35%, depending on the type. been found to have higher apparent diffu- toid odontogenic tumor is one of the few le-
Solid (multicystic) ameloblastomas, pre- sion coefficient values compared with kera- sions that favor the anterior maxilla over the
viously referred to as adamantinomas of tocystic odontogenic tumor [42]. This find- posterior mandible. The typical radiographic
the jaw, constitute over 80% of all amelo- ing presumably reflects that the cystic spaces appearance of adenomatoid odontogenic tu-
blastomas. Multicystic ameloblastoma is a of ameloblastoma are less densely packed mor is a unilocular corticated radiolucency
slow-growing locally aggressive neoplasm and viscous compared with the accumulat- around the crown, which can extend inward
that originates from odontogenic epitheli- ed keratin in a keratocystic odontogenic tu- to involve the root of the tooth.
um. It generally affects adults, with a mean mor. Other MRI parameters have been inves- The histologic profile of adenomatoid odon-
age of onset of 33 years, and is rare in pa- tigated to discriminate cystic ameloblastoma togenic tumor shows epithelial cells arranged
tients younger than 12 years. About 85% of from other causes, including dynamic con- in two patterns: some are spindle shaped, ar-
solid ameloblastomas occur in the posterior trast enhancement, signal uniformity, and T2 ranged in whorls, nests, and bundles, whereas
mandible in the molar-ramus area [35]. The relaxation times [29, 42, 43]. others are cuboidal and arranged in ductlike
radiologic appearance of solid ameloblasto- structures, with the epithelial proliferations
ma comprises a well-corticated, expansile, Odontogenic Myxoma supported by a thick fibrous connective tissue
multilocular, and radiolucent lesion, often Fibromyxoma or myxofibroma, better capsule. Globules of a homogeneous materi-
accompanied by enhancing soft tissue [33] known as odontogenic myxoma, is an un- al, which may represent amyloid, have been
(Fig. 6A). Solid ameloblastoma often has a common neoplasm of odontogenic mes- described in some adenomatoid odontogenic
nonaggressive appearance on radiographs enchymal origin [44]. Although they are tumor specimens. The thick connective tissue
and may be indistinguishable from other benign, odontogenic myxomas, like amelo- capsule makes separation of the lesion from
odontogenic lesions, although more aggres- blastomas, may be locally aggressive, with a the tooth and surrounding connective tissue
sive features, such as resorption of adjacent potential for extensive bony destruction and easy, allowing the clinician to save the tooth.
roots, may suggest the diagnosis. If left un- extension into surrounding structures [45]. Therefore, the treatment of choice is either
treated, solid ameloblastoma, even if other- Almost 75% of odontogenic myxomas are enucleation or simple curettage.
wise asymptomatic, can enlarge substantial- diagnosed in young adults 23–30 years old.
ly, causing facial disfigurement. At least five Odontogenic myxomas occur only rarely in Nonodontogenic Lucent Lesions
histologic subtypes of solid ameloblastoma patients younger than 10 years. These lesions Simple Bone Cyst
have been described, all appearing as vari- are found almost equally in the maxilla and Simple bone cysts of the same type that
ably shaped and sized odontogenic epitheli- mandible, showing a slight predilection for may arise in the proximal humerus (50%)
al islands, surrounded by mature and cellu- the posterior mandible. When they occur in and femur may also arise in the mandible.

4 AJR:208, January 2017


Benign Pediatric Mandible Disorders

Although some simple bone cysts may be the temporomandibular joint [56]. The radio- hazardly distributed giant cells of variable
related to prior trauma, which explains why graphic appearance of ABCs can be variable shapes and sizes (Fig. 8C). Extravasated
simple bone cysts are sometimes referred as well, but they are most commonly seen as erythrocytes with hemosiderin deposition
to as traumatic bone cavities, other possible multilocular radiolucencies with cortical thin- are commonly present. On gross examina-
causes for simple bone cysts include cystic ning leading to ballooning or blown-out dis- tion, the dark brown color of giant cell gran-
degeneration of a preexisting tumor, loss of tention of bone [57] (Fig. 7). If MRI is per- uloma may resemble liver.
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the fatty marrow in the involved area, or vas- formed, ABCs will typically show multiple
cular abnormality resulting from high ve- fluid-fluid levels secondary to the sediment of Other Lesions
nous pressure in the medullary space [48– blood products that is characteristic of these Multiple other lesions that occur elsewhere
51]. Simple bone cysts are most commonly lesions. Of note, fluid-fluid levels can occur in in the body can also present in the mandible.
discovered in the second decade of life [48]. other lesions of the jaw, including osteosarco- These include intraosseous vascular malfor-
The most common location for simple bone ma and fibrous dysplasia [58, 59]. mations and chondroid lesions [67–69]. Non-
cyst [49] is the posterior mandible, particu- ossifying fibromas are rare in the mandible
larly the premolar-molar region. If locat- Central Giant Cell Granuloma but can be multiple in the context of Jaffe-
ed anteriorly, they can sometimes cross the Central giant cell granuloma of the jaw is a Campanacci syndrome [70]. Finally, the mel-
midline. Those arising in the premolar-molar separate entity from the giant cell tumor seen anotic neural ectodermal tumor of infancy
area are typically located above the alveolar in long bones, despite their similar names. is a rare neoplasm of neural crest cells that
canal, usually are unilocular and radiolucent, Although these lesions may have a similar presents in the first year of life. It most com-
and, in many cases, have a scalloped superior radiologic appearance, they are distinct enti- monly affects the maxilla and skull but can
border insinuating between the roots of teeth. ties histologically [60]. It is unclear whether also involve the mandible [71, 72].
The associated teeth may, in fact, be found giant cell granuloma represents a neoplastic
dangling within the cavity. The 25% of the or reactive process, and the variable opin- Conclusion
lesions occurring in the anterior mandible, ions about cause are reflected in the variety The differential diagnosis of a lucent man-
apical to the canine tooth, are usually round of names for this entity. The term “giant cell dibular lesion is broad, because there is a
and unilocular radiolucencies with an ap- granuloma” will be used in the current arti- broad spectrum of both odontogenic and
pearance similar to that of periapical cysts. cle for simplicity [61]. Although giant cell tu- osseous pathologic abnormalities that can
In contrast to simple bone cysts of the long mors typically occur in the long bones, giant manifest in this fashion. Although the imag-
bones, most simple bone cysts that arise in cell granulomas occur most commonly in the ing appearance of these lesions can be quite
the mandible are asymptomatic, and patho- mandible and maxilla, followed by the digi- similar, using the location and the provid-
logic fractures are uncommon. At surgery, tal bones. Giant cell granulomas are indistin- ed clinical history can help tailor the differ-
an empty cavity is found at the cyst entrance guishable from cherubism and brown tumors ential considerations. It should also be not-
in about two-thirds of cases, and cavities of hyperparathyroidism histologically, and ed that, in addition to the benign but locally
filled with straw-colored fluid are observed are therefore simply called central giant cell aggressive neoplasms discussed here, both
in about one-third of cases. The treatment lesions by many pathologists. Twice as many odontogenic and nonodontogenic malignan-
of choice for this condition is scraping the cases of giant cell granuloma occur in female cies can present as a lucent lesion as well.
bone cavity to generate bleeding, but other patients as compared with male patients, and
accepted treatments include the injection of over 60% of giant cell granuloma cases are References
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Fig. 1—6-year-old boy


with dentigerous cyst.
A and B, Axial (A)
and sagittal (B) CT
images show unilocular
expansile lucent lesion
(arrows, A) with intact
cortex. Note cyst’s
association with crown
of unerupted tooth
(black arrow, B) and
absorption of roots of
adjacent teeth (white
arrow, B).
A B (Fig. 1 continues
on next page)

AJR:208, January 2017 7


Plunk et al.

Fig. 1 (continued)—6-year-old boy with dentigerous cyst.


C, Histologically, dentigerous cysts are simple epithelium-lined unilocular
cystic lesions surrounded by connective tissue wall. Usually, they are lined by
nonkeratinizing squamous epithelium, but occasionally columnar or cuboidal
epithelium, or mucous-producing cells, are prominent component of cyst lining.
Photomicrograph (H and E, ×200) shows that connective tissue is infiltrated by
lymphocytes and plasma cells.
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A B

C D
Fig. 2—14-year-old boy with dentigerous cyst.
A and B, Axial (A) and coronal (B) CT images show expansile lucent lesion in left mandible with thinned but otherwise intact cortex (arrows), associated
with crown of unerupted tooth.
C, Coronal STIR image shows uniformly high-signal simple fluid within cyst (arrow).
D, Axial T1-weighted fat-suppressed contrast-enhanced MR image illustrates thin marginal enhancement (arrows).

8 AJR:208, January 2017


Benign Pediatric Mandible Disorders
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A B
Fig. 3—13-year-old boy with periapical (or
radicular) cyst.
A and B, Axial (A) and sagittal (B) CT
images show ovoid cyst with intact cortex
(arrows). Sagittal image (B) shows cyst’s
association with root of anterior incisor.
C, Photomicrograph (H and E, ×100)
shows that this periapical cyst has
typical nonkeratinizing squamous lining
surrounded by inflamed hemorrhagic
connective tissue wall, infiltrated
by sheets of foamy macrophages,
lymphocytes, and plasma cells. Histologic
profile of periapical cyst is similar to that
of dentigerous cyst. Clinical and imaging
correlation is needed for definitive
diagnosis.

AJR:208, January 2017 9


Plunk et al.
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B C
Fig. 4—20-year-old man with
keratocystic odontogenic tumor.
A, Panoramic radiograph depicts
multiloculated lytic lesion (black
arrow) extending longitudinally
along left mandibular body (white
arrows).
B and C, Axial CT images reveal
expansion and thinning of cortex
(arrows, B), greatest laterally where
it protrudes into overlying soft
tissues (arrow, C).
D, Photomicrograph (H and E, ×100)
shows that these cysts are lined by
stratified squamous epithelium with
corrugated surface. Basal cells are
hyperchromatic and have palisaded
appearance. Cystic spaces may be
filled with amorphous keratin debris.
These diagnostic histologic features
may be lost in cases where cyst
becomes inflamed.
D

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Benign Pediatric Mandible Disorders
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A B
Fig. 5—17-year-old girl with ameloblastoma (unicystic).
A and B, Axial contrast-enhanced CT images show large septate cystic lesion
centered within right posterior mandibular body, with enhancing soft tissue along
cyst’s anteromedial aspect (arrowheads, A; arrows, B).
C, Ameloblastomas are composed of multiple variably sized islands of epithelium
in backgrounds of fibrovascular tissue. Characteristic features include basal
columnar cells with reversed nuclear polarization and central zones of loose
stellate epithelium, as seen on photomicrograph (H and E, ×400).

A B
Fig. 6—20-year-old woman with ameloblastoma (solid).
A, Axial contrast-enhanced CT image illustrates enhancing soft tissue (white arrow) in central portion of left mandibular lytic lesion. Note erosion through lateral cortex
(black arrow).
B, Photomicrograph (H and E, ×200) shows typical histologic features of solid ameloblastoma. There are odontogenic epithelial islands of variable shapes and sizes.
Islands are lined by single layer of palisaded columnar cells exhibiting reverse polarization. Center of islands is filled with stellate reticulum type of epithelial cells.
Connective tissue stroma is mature and cellular.

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Plunk et al.
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A B
Fig. 7—18-year-old man with aneurysmal bone cyst.
A and B, Axial (A) and coronal (B) contrast-enhanced
CT images show multicystic expansile lesion in left
mandibular ramus and condyle (arrows, B). Fluid
exhibits variable attenuation above that of water,
reflecting proteinaceous contents or blood products,
including fluid-fluid level (arrow, A).
C, Aneurysmal bone cysts are composed of irregular
pseudocystic spaces that may contain blood. Fibrous
septa in aneurysmal bone cysts contain bland
fibroblasts, capillaries, multinucleated giant cells
without cytologic atypia, and seams of osteoid. Mitotic
figures may be prominent focally, but atypical mitotic
figures are not seen in photomicrograph (H and E, ×200).

A B
Fig. 8—10-year-old girl with central giant cell granuloma.
A and B, Axial (A) and coronal (B) CT images depict expansile lucency in right parasymphyseal mandible (arrows). Overlying cortex is very thin
and has been fully eroded in parts, giving lesion aggressive appearance.
(Fig. 8 continues on next page)

12 AJR:208, January 2017


Benign Pediatric Mandible Disorders

Fig. 8 (continued)—10-year-old girl with central giant


cell granuloma.
C, Photomicrograph (H and E, ×200) shows numerous
osteoclastlike multinucleated giant cells admixed
with spindled and cellular granulation tissue. Patchy
regions of hemorrhage are common.
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AJR:208, January 2017 13

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