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The term odontogenic implies being derived from development (2). Odontogenic cysts can become inflamed
tooth-forming structures. Odontogenic cysts and tumors or infected, causing significant signs or symptoms.
are a unique group oflesions due to their complex and vari-
able history, histologic characteristics, and clinical behav- INFLAMMATORY CYSTS
ior. Odontogenic cysts vary significantly in their frequency,
behavio~ and treatment. Odontogenic tumors are rela- Radicular Cyst
tively uncommon lesions (1). Collectively, lesions of the The radicular cyst is by far the most common odontogenic
jaws deserve considerable attention by surgeons because of cyst. It develops at the apex of an erupted tooth in response
their potential for tissue destruction and the related chal- to pulpal necrosis secondary to dental caries or trauma.
lenges they pose for reconstruction. This chapter reviews The cyst arises from inflammatory stimulation and prolif-
the more common odontogenic cysts and tumors along eration of the rests of Malassez. The cyst lining forms as
with several other related jaw lesions. The salient clinical, the epithelial elements proliferate. Cellular debris within
radiographic, and histologic features of each lesion will the lumen produces an osmotic gradient. and fluid is trans-
be discussed, in addition to its treatment and prognosis. ported across the lining. This gradient slowly increases the
Many of the odontogenic cysts and tumors share similar volume of fluid inside the lumen, ultimately expanding
radiographic features, so the patient's history and eventual the cyst by hydraulic pressure from within the cyst.
histopathology ultimately dictate the specific surgical ther-
apy. Furthermore, a frozen section is not typically obtained Clinical and Radiographic Features
for jaw lesions discussed in this chapter because they are Most radicular cysts are asymptomatic and are discov-
not malignant lesions and a frozen section will not change ered incidentally during routine radiographic evaluation
the ultimate treatment plan for each lesion. (Fig. 132.1). Radicular cysts rarely exceed 1 em in diameter
except when several adjacent teeth become devitalized as
a result of trauma. Radiographically, the radicular cyst is
ODONTOGENIC CYSTS round to ovoid, well circumscribed, and contiguous with
the apex of the involved tooth.
In 1992, the World Health Organization (WHO) classified
odontogenic cysts as either inflammatory or developmen- Histopathology
tal, including odontogenic and nonodontogenic. Most The cyst is lined by stratified squamous epithelium of
cysts of the oral cavity are true cysts because they contain varying thiclmess. The cyst wall typically supports a vari-
an epithelial lining. The lining of these cysts is derived from able inflammatory cell infiltrate, including lymphocytes
one of three epithelial structures: (a) reduced enamel epi- and neutrophils. The cystic lumen will frequently con-
thelium-residual epithelium that surrounds the crown of tain necrotic cellular debris. A small percentage of radic-
the tooth after enamel formation is complete; (b) rests of ular cysts have crescent-shaped hyaline (Rushton) bodies
Malassez-remnants of the Hertwig root sheath that persist within the epithelial lining. Although unique to odonto-
in the periodontal ligament after root formation is com- genic cysts, the biologic significance of Rushton bodies is
plete; or (c) remnants of dental lamina (rests of Serres)- unknown. Multinucleated foreign body giant cells, choles-
islands and strands of epithelium that originate from the terol crystal clefts, and hemosiderin may be seen through-
oral epithelium and remain in tissues after inducing tooth out the connective tissue of the cyst wall
2097
2098 Section VII: Head and Neck Surgery
DEVELOPMENTAL CYSTS
Dentig4H'OUS Cyst
The dentigerous cyst is the second most common odon-
togenic cyst A dentigerous cyst must be associated with
the crown of an unerupted tooth (3). The cyst is attached
to 1he cerrical region of the too1h, usually a mandibular
third molar. However, variants crist where the cyst is seen
lateral to or completely enveloping the associated tooth.
Maxillary third molars, maxillary canines, and mandibu-
lar second bicuspids are also commonly involved, as these
c
Figure 132.2 A,B: Dentigerous cyst. Panorex radiograph and
teeth are among those frequently impacted. computed tomography (Cl) scan of 14-year-old who presented
with facial swelling. Radiograph reveals considerable left mandibu-
Clinical and Radiographic Features lar angle and ramal involvement. Note thinning of bucxal and lin-
gual cortices and displacement of multiple permanent teeth within
Dentigerous cysts usually occur in the second and third lesion on CT. C: Surgical specimen from above patient. Note
decades with a slight male predilection. Because they are adherence of cyst to permanent tooth crown.
associated with an impacted tooth, the arch will appear to
be missing a tooth. The cysts usually do not produce symp- cysts can displace 1he tooth into the ramus or the inferior
toms and can achieve significant size, causing bony expan- border of the mandible, while maxillary cysts can displace
sion (Fig. 132.2A and B). The dentigerous cyst is usually a the tooth into the maxillary sinus toward the o:rbital floor.
well-ciralmsaibed, uniloculat radiolucency surrounding It is important to understand that the above-stated clini-
the crown ofan unerupted tooth (Fig. 132.2C). Mandibular cal and radiographic features are not specifically diagnostic
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2099
Histopathology
The cystic lumen is lined bynonkeratinized, stratified squa-
mous epithelium that is anywhere from 2 to 10 cell layers
thick. It is common for the lining to be inflamed. The den-
tigerous cyst can share several microscopic features with
the radiailar cyst, such as Rushton bodies, cholesterol crys-
tal clefts, and hemosiderin deposits. Chronic dentigerous
cysts may exhibit areas of keratinized epithelium, which
require the lesion to be differentiated from the KCOf (5). Figure 132.3 Calcifying odontogenic cyst (Gorlin cyst). Cyst
The epithelial lining of the dentigerous C}'!t is capable of lined by odontogenic epithelium. The basal calls are palisaded and
columnar. Large, eosinophilic "ghon cells• are seen within and on
ameloblastoma expression (3). Thus, timely diagnosis and the surfaca of the epithelium.
treatment is critical.
Histopathology Histopathology
These usually unilocular cysts are lined by odontogenic this lesion has several unique microscopic features: (a) an
epithelium. The basal layer is distinct with its columnar to epithelial lining of variable thidmess and a flat epithelial-
cuboidal cells with hyperchromatic nuclei that are polarized connective tissue junction; (b) small micro cysts and gland-
away from the basement membrane (Fig. 132.3). Another like structures within the epithelial lining; and (c) a single
characteristic histologic feature of the COC is the ghost layer of cuboidal or columnar cells lining the gland-like
cell-an altered epithelial cell with loss of the nucleus and structures (F'tg. 132.4).
2100 Section VII: Head and Neck Surgery
Odontoma
Odontomas are not ttue neoplasms, but rather hamarto-
matous growths because they form during normal tooth
development and then reach a fixed size. The lesion con-
tains elements of enamel, dentin, cementum, and pulp
A
tissue. Depending on the degree of morphologic differenti-
ation. an odontoma can be classified as either compound,
if the lesion resembles tooth-like structures or complex. if
the lesion appears as an amorphous mass.
Histopllthology
.Although reduced enamel epithelium may be present.
odontomas are essentially composed of enamel, dentin,
cementum, and pulp tissue. Fibrous tissue is sparse.
B
Treatment and P10gnosis Figure 132.5 A; Keratocystlc odontogenic tumor. Panoramic
Removal of the lesion may be necessary to rule out other radiograph of a 18-year-old man w!th bllm~ral mandibular and
lesions or if the mass is impeding the eruption of a tooth. maxillary KCOT. Patient also had NBCCS. 1: Axial compuNd
Enucleation and curettage is considered curative,. and the tomography (CT) scan of same patient, revealing bilateral rnaxll·
lary lesions. Lesions "-"''re noted to extend to the orbital ftoors
lesion is not known to recur. Intraoperatively.
B D
E F
Figure 132.6 A:. Ameloblastoma. Panoramic radiograph of a 17-year-old girl with unilocu-
lar amtilloblastoma of right mandible. Note resorption of multiple tooth roots In right mandible.
B: Coronal comp~m!d tomography (CT) scan of maxillofacial bones of patient from (A). Note
extremtil buccal and lingual cortical expansion of right mandible. Focal areas of perforation 'Mire
not:ad Intraoperatively. C: Ameloblastoma (follicular pat:t8m}. Islands of odontogenic epithelium char-
acterized by peripheral columnar a~lls exhibiting revern polarity. The central portion of these Islands
resemble stellma retiC1Jium In areas. D: ArMioblastoma (plexiform pattern). E: Preoperative photo
of patient from previous Images. Note significant vestfbular expansion. Right mandibular teeth
exhibited significant clinical mobility. F: A resection of the right mandible from the angle to the
contralateral parasymphy5is through a neck incision. Focal areas of buccal cortical perforation were
noted intraoperatively. Specimen is viErWed from the buccal aspect.
2104 Section VII: Head and Neck Surgery
from Hertwig root sheath. These lesiolll!l can be mistaken prominent amyloid presence is another unique character-
for ameloblastoma by an inexperienced pathologist If the istic feature of the CEOf (37).
demographic and radiographic features are not colll!listent
with a diagnosis of ameloblastoma, a second opinion Treatment and Prognosis
should be sought Much like the ameloblastoma, the CEO!' is treated by resec-
tion, but no cwrent study has stated an adequate bony mar-
TrHtment and Prognosis gin to achieve a cure A minimum of a 1.0-an. bony mcugin
Recurrence is not seen in this benign encapsulated lesion, along with any necessaif soft tissue to still achieve one layer
so enucleation and curettage alone is curative. If the cap- of an uninvolved anatomic barrier is recommended unless
sule is inflamed and becomes compromised during enucle- determined otherwise by a frozen section. Recw:rence has
ation of the lesion, the surgeon should thoroughly inigate been reported, so long-term follow-up is recommended.
the surgical field. Bony regeneration of the defect usually The peripheral variant is treated by local excision with a
occurs in about 1 year in younger patients. 5-mm margin, which should include the underlying peri-
osteum. The remaining wound may be closed either pri-
marily or by local advancement ftaps.
Calcifying Epithelial Odontogenic Tumor
(CEOT, Pindborg Tumor)
Odontogenic Myxoma
The calcifying epithelial odontogenic tumor {CEOf) is a
rare odontogenic tumot representing less than 1% of all The odontogenic myxoma, although a mre tumor;. is
odontogenic tumOIS. It originates from the epithelial rests of worthy of mention due to its locally aggressive behavior
the dental lamina or reduced enamel epithelium. The CEOI' and high reau:rence rate due to its infiltrative nature In
has distinct histologic features and a variable radiographic the jaw bones, it is derived from odontogenic ectomesen-
appearance. Like the ameloblastoma, it is considered a chyme that is very similar to the mesenchymal tissue of the
locally aggressive neoplasm that can be highly infiltrative follicular sac or dental papilla. The odontogenic myxoma
and destructive and must be surgically excised {33,34). only mrely ocaus in non-tooth-bearing portiolll!l of the
jaws or other facial bones (38).
Clinical and Radiographic Features
The CEO!' has been reported in patients over a wide age Oinical and Radiographic NHitures
range. but has a peak incidence in the fifth decade It pres- The odontogenic myxoma has some clinical and radio-
ents as a slow-growing, fum, painless swelling usually in graphic features in common with the ameloblastoma. It is
the posterior mandible The molar area is the most frequent usually asymptomatic and slow growing. It rarely displaces
region in either jaw. Like most other benign odontogenic teeth or resorbs roots and does not alter sensory function.
tumors, the CEOT does not alter nerve function. A periph- Although it has been reported over a wide age range, it is
eral variant exists and typically presents as a soft tissue swell- most commonly seen in the third decade, which is slightly
ing in the anterior aspect of the mouth (35). Most of these younger than the peak occwrence of the ameloblastoma. The
lesions appear as diffuse radiolucent lesions, unless they are lesion has been reported in all portions of both jam, but usu-
laxge or mature; then they will exhibit areas offaint opacities ally oa::u:m in the posterior mandible Radiographically, the
colll!listent with the presence of calcificatiolll!l in the lesion. It lesion is radiolucent and more often multilorulat but can be
is typically associated with the crown of an impacted tooth. unilOOJlar (Fig. 132.8). The lesion also has been described
Radiographic borders are variable, mnging from distinct as having a "honeycomb• appearance, with its faint. wispy
lines to diffuse opacified areas fusing with adjacent bone ttabeailar bone mixed within cortical plate displacement
Histopathology
The most common pattern of the CEOI' is one of sheets or
strands of polyhedral epithelial cells connected by inter-
cellular bridges. The nuclei have prominent nucleoli, and
the cells may even appear pleomorphic. but this does not
imply a malignant state, and mitotic figures are not rou-
tinely found. Scattered calcifications are a distinct feature
of the CEOf, and the calcifications form concentric rings
referred to as Liesegang rings. Another histologic variant
containing clear cell features has been described and asso-
ciated with increased clinical aggressiveness (36). Large
areas of amorphous eosinophilic material are dispersed
Figure 132.8 Odontogenic myxoma. Panoramic radiograph
throughout the lesion. These areas stain positive for amy- revealing diffuse, miud radlolucent/rad!opaquelnvolvei'I'Kint of left
loid with Congo red, aystal violet. or thioflavin T. This hemlmand!ble. Lesion extends from midline up to sigmoid notch.
2106 Section VII: Head and Neck Surgery
Histopathology
The odontogenic myxoma is an infiltrative and gelati-
nous tumor. It contains scant. randomly arranged spin-
dle-shaped mesenchymal cells within a myxoid ground
substance, which stains predominantly basophilic with
hematoxylin and eosin. When these lesions have a more
significant collagenous component. they are referred to as
myxofibromas or :fibromyxomas. How~ this dassi:fica-
tion does not alter the lesion's clinical behavior.
•
•
'
.. •
•
Clinical and Radiographic Features irregular bony trabeculae. Fibrous dysplasia most com-
The 1Jpical patient with an ossifying fibroma ia a woman in monly occurs in one bone (monostotic), or more rarely
her second to fourth decade, although the lesion has been multiple bones (polyostotic). Polyostotic fibrous dysplasia
reported over a wide age range and in both sexes. Immature may be seen as a component of McCune-AllTright syndrome,
lesions initially present as a radiolucency, but become more which includes caft-au-Iait skin macules, and multiple
mixed as they mature and eventually can become com- endocrinopathies, including hyperthyroidism and/or pre-
pletely radiopaque. More aggressive lesions expand the cor- cocious puberty (43). Although genetically based, it is
tices of the jaws and frequently displace adjacent structures. usually a sporadic condition involving a mutation in the
In the mandible, they typically appear as a mid-body growth a-subunit of a signal-transducing G protein.
at the inferior borda; enlcuging outward and downward as
if "hanging off the lower lateral border" of the mandible. Clinical and Radiographic Features
Fibrous dysplasia is a slow-growing, f1pically asymp-
Histopathology tomatic process that often produces a bony hard swell-
Ossifying fibromas are well demarcated from the sur- ing (Fig. 132.13A-C). The condition can be self-limiting,
rounding bone and are composed of dense cellular fibrous beginning in the tmt decade and ceasing when the affected
tissue with variable amounts of calcified trabeculae of oste- bone readtes maximum growth and maturation. It is capa-
oid or bone and/or spherical cementum-like structures ble of tooth displacement with resultant malocclusion.
(Fig. 132.12). The ossifying fibroma ia usually well circum- Lesions located in the paranasal sinuses can cause nasal
scribed and does not exhibit diffuse infiltration of adjacent obstruction. Fibrous dysplasia involving the midface,
tissues, unlike fibrous dysplasia. calvarium. or skull base can cause progressive severe facial
distortion and cranial nerve dysfunction via compression.
Treatment and P10gnosis In such individuals, serial visual acuity and audiology test-
These lesions are amenable to enucleation and curettage if ing correlated with 1-mm cut computerized tomography ia
detected early, or resection for lcuger lesions. Because ossi- warranted to monitor progression and guide timing of any
fying fibromas do not display aggressive infiltration of sur- necessaJ:Y surgical intervention. Early lesions are radiolu-
rounding tissues, for those requiring surgical resection due cent but become more opaque as the lesion matures. The
to large size or problematic location, a consenative 5-mm normal medullary bone is replaced with a fine trabecular
ma!gin is appropriate, and recurrence is rare. The juvenile bone, giving the lesion its •ground-glass• appearance on
(aggressive) ossifying fibroma is a rare variant of the above radiographs. Fibrous dysplasia does expand the cortices
and ia considered a more aggressive lesion. appearing at a but does not displace the inferior alveolar canal. The mar-
younger age with a predilection for the maxilla. gins of the lesion are usually not well demarcated.
Histopathology
Fibrous Dysplasia Fibrous dysplasia is seen microscopically as irregularly
Fibrous dysplasia is not a true neoplasm; rathe:t it is a shaped trabeculae of woven bone in a background of a loose
genetic. tumor-like condition where normal medullary and cellul~ fibrous connective tissue (Fig. 132.130). Fibrous
bone is replaced with fibrous connective tissue mixed with dysplasia has a distinctive mi.aoscopk picture of abnormal
bone coalescing with the nonnal surrounding tissue, which
is in contrast with the featu.res of the ossifying fibroma.
those that appear in long bones, but the biologic behavior Aneurysmal Bone Cyst
differs. The CGCL is an osteolytic lesion that has been
referred to as a central giant cell reparative granuloma in The aneUiysmal bone cyst (ABC) is a rare, expansile osteo-
the past, but current understanding is that it does not rep- lytic bone lesion. It contains large blood-filled spaces that
resent a reparative process ( 45,46). do not have an endothelial or epithelial lining, and is thus
not a true cyst.
c
B
Figure132.14 A:. Cemnl giant call lesion. Panoramic radiograph
demonstrating upansive ill-defined lesion at right angle of man-
dible. Patient notia~d a gradually inaeasing swelling over the right
mandible, while her ocxlusian was shifting to the left. B: CGCL
Numerous muhinuc:leated giant calls in a background of plump, prim-
itive mesenchymal eel!f. Abundant hemorrhage is seen thro~out.
C: A right hemimandibulectomy was performed via a right subman-
dibular approach. The specimen dernonstnrte9 the well-vascularized
appearance of the ICISion. D: Post-op panoramic radiograph reveals
reconstruction with an lilac mast bone graft. Endosseous Implants
""""'na pii.IOiild 7 months aftQr Initial graft. D
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clinical and ultrastructural study. Oral Surg 1975;39:901-917. lesions of the jaws: a clinicopathologic study. 1 Oral MaxiUofac
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