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William L. Chung Kurt F. Summersgill Mark W.

Ochs

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

Flgwe132.1 Periapical cyst. Raciograph demonstrating periapical


lesion associated w!th grossly c:arlous tooth I 20.

Treatment and P10gnosis


1hese cysts are treated by extraction of the infected tooth
followed by enucleation of 1he cyJt. Extraction of the tooth
wi1hout removing 1he cyst may allow for persistent growth
of the cysllf the cyst is incompletely removed, a residual cyst B
may develop. Complete bony healing is typially seen within
6 months if1he radiOJlar cyst has been 1horoughlyremoved.

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

for a dentigerous cyst. although highly consistent {4). The


differential diagnosis for similar-appearing radiographic
lesions should include an ameloblastoma. keratocysti.c
odontogenic tumor (KCOI'), and adenomatoid odonto-
genic tumor (Aar).

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.

TnN~tment and Prognosis bright eosinophilic cytoplasm-that represents abnormal


Removal of the impacted tooth along with thorough enu- keratinization. With time, these ghost cells tend to become
cleation of the C}'!t is usually definitive therapy. When a calcified, sometimes even forming calcified masses. When
mandibular dentigerous C}'!t reaches considerable size, the these cells contact the connective tissue, a foreign body
cyst can be marsupialized to allow for decompression and reaction ocrurs due to the release of keratin.
shrinkage of the cyst with compensatoty bone :fill prior to
definitive removal of the lesion.
Treatment and Prognosis
Swgical enucleation usually results in complete resolution.
Calcifying Odontogenic Cyst (Gorlin Cyst) The emaosseous lesions are often associated with other
odontogenic tumors and due to their low recurrence
This lesion is thought to arise from remnants of the dental
potential are managed conservatively with lesion removal
lamina within the gingiva or jaws. Approximately one-
only.
fourth of all calcifying odontogenic cysts {COGs) occur
emaosseously, within the gingiva anterior to the :first
molar in individuals older than 50 years (6).
Glandular Odontogenic Cyst
(Sialo-Odontogenic Cyst)
Clinical and Radiographic Features
The COC behaves like most other odontogenic cysts, and The glandular odontogenic cyst was first described in
it has little recurrence potential. The COC has a wide 1987 (7). Although only a few cases have been reported, it
age range, but the peak incidence occurs in the second is worth mentioning due to its locally aggressive behavior
decade. The lesion has a predilection for females, and and its propensitr to recur. This lesion is generally consid-
most are located in the anterior portion of either jaw. The ered odontogenic in origin with numerous mucus-secret-
extraosseous lesion may present with painless gingival ing cells within its epithelial lining.
expansion. These cysts are often discovered as incidental
:findings on routine radiographic evaluation. They ini- Clinical and Radiographic Features
tially appear lucent. As the cyst develops, calcifications this lesion occurs most commonly in middle-aged adults.
may develop causing a well-circumscribed mixed radio- The majority of glandular odontogenic cysts have been
lucent/radiopaque appearance. Howeve~ these opacities reported in the mandible especially in the anterior region.
are only seen in approximately one-third to one-half of they appear as multilocular radiolucencies and may cross
all cases. the midline.

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

with the nasal cavity ( dliated columnar). The fibrous tissue


contains elements of nerve and vasrular tissue comis-
tent with the surrounding incisive canal contents. Mucus
glands are occasionally seen within the wall of the cyst as
these glands are native to the adjacent nasal cavity.

TnNtment and Prognosis


Surgical enucleation is usually curative. but may result in
sacrificing the nasopalatine nerve and vessel, resulting in
denenration of the mucosa of the anterior palate and max-
illaxy incbont. Reaurence is rare

Stafne Bone Cyst (Lingual Salivary Gland


Depression, Static Bone Cyst)
Figure 132.4 Glandular odontogenic cyst. Stmified squamous The Stafne bone cyst is not a ttue cyst Rath~ it is a depres-
epithelium forming gland·like stRictures.
sion on the lingual aspect of the posterior body of the
mandible This condition has a pathognomonic radio-
graphic appearance of a small ovoid, well-circumscribed
Treatment and P10gnosis
Most glandular odontogenic cysts are amenable to enucle- radiolucency often surrounded by cortical bone. located
below the inferior alveolar canal in the second or third
ation and curettage. Howevet due to its recurrence poten-
tiaL some advocate mcuginal resection (8). Regardless, molar region. This radiographic appearance is due to the
relative thinning of the mandible. Most reported cases
long-tenn follow-up is advisable
have occurred in males. Sialography, biopsy, and autopsy
have revealed that the depression is characteristically filled
NONODONTOGENIC CYSTS with an accessory lateral lobe of the submandibular sali-
vary gland, although occasionally connective tissue. adi-
Nasopalatine Duct Cyst (Incisive Canal Cyst)
pose tissue. and lymphoid tissue have been seen {10). No
The nasopalatine duct cyst originates from the epithelial treatment is required.
remnants of the two embtyOnic nasopalatine ducts (9).
The majority of these cysts arise in the anterior maxilla
Idiopathic Bone Cavity (Traumatic Bone
near the incisive foramen.
Cyst or Traumatic Bone Cavity)
Clinical and Radiographic Features The ttaumatic bone cavity is not a ttue cyst because it does
Men are affected twice as often as females, and this cyst not contain a true epithelial lining. It is usually found in
ocrurs most frequently in the fourth to sixth decades. The the posterior mandible and presents on radiograph as a
nasopalatine duct cyst is either asymptomatic or presents less well-defined radiolucency compared to most odonto-
as a soft tissue swelling in the midline of the anterior hard genic cysts. 1hese lesions are typically empty on swgical
palate once the lesion has perforated the bone The lesion exploration or contain some sttaw-colored fluid. There is
is typically a well-drcumsaibed, oval-pear or heart-shaped, no lining or tissue to submit for biopsy, but exploration
unilorular radiolucency in the midline of the anterior pal- of the lesion will be curative, since hemorrhage within
ate. If the cyst readtes large proportiom, it can resoib the the cavity will allow for granulation tissue formation and
roots of the adjacent teeth. Some normal incisive canals are ultimate resolution of this condition.
radiographically wide. which may make diagnosis of this
lesion less precise. It is generally accepted that any radio- ODONTOGENIC TUMORS
graphic lesion greater than 6 mm should be comidered a
cyst and not an enl;uged incisive foramen. Ifno soft tissue or Odontogenic tumont are collecti'ftly a rare but diverse
bony swellings are present and the patient is asymptomatic, and complicated group of lesions. They arise from epi-
then a diagnosis of nasopalatine duct cyst becomes unlikely. thelial or mesenchymal cells, or both, that are associated
with tooth structures. Most odontogenic tumors are ttue
Histopathology neoplasms, but some behave as hamartomatous growths.
The lining of this lesion can be either stratified squamous Odontogenic tumors commonly present as asymptomatic
epithelium, dliated columnar or cuboidal epithelium, swellings, which can eventually cause bone loss, tooth dis-
or a combination of the two. This variable lining reflects placement,. and jaw apamion. They rarely cause sensory
whether the cyst has arrived from epithelium closer to the nerve dysfunction. Understanding the biologic behavior of
palate (sttatified squamous) or more closely associated this group oflesions will assist in choosing the appropriate
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2101

treatment to best attain a cure or to optimize the outcome


(11,12). The tumors discussed in this chapter were selected
based upon their frequency, locally aggressive behaviot
andfor likelihood of recurrence

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.

Clinical and Radiographic Features


1he odontoma is the most common odontogenic •tumor. •
The lesion is tJpically asymptomatic and is discovered in
the first two decades on radiographic examination, often
initiated to evaluate lack of expected eruption of a per-
manent tooth. Radiographically, both the compound and
complex odontomas are radiopaque masses and have a
well-demarcated border. The compound odontoma resem-
bles multiple tiny tooth structures, while the complex
odontoma appears as a dense irregular 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.

Keratocystic Odontogenic Tumor


Oinic:al and Radiographic Features
(Odontogenic Keratocyst) The KCOf differs from other odontogenic lesions with
The KCOf is a Wli- or multicystic, intrabony lesion of regard to its growth potential and recurrence rate. It can
odontogenic origin. It develops from remnants of the display aggressive growth, causing bony expansion and
dental lamina and can occur in any location of the jaws destruction, and reports have shown recurrence rates from
(13,14). Its previous traditional term, odontogenic ker- 5% to 60% (16,17). The KCOf occurs over a wide age
atocyst (OKC), emphasized the benign behavior of this range; however, its peak incidence is within the second and
lesion. However;. in 2005, the WHO Working Group thin:l decades. When multiple KCOTs occur in a patient
recommended the newer term KCOf because it more the nevoid basal cell carcinoma syndrome (NBCCS) must
accurately described its neoplastic behavior (15). These be considered. 1he mean age of patients with multiple
lesions mimic the radiographic appearance of any other KCOTs, with or without NBCCS, is lower than those with
odontogenic cyst and some odontogenic tumors. The single, nonrecurrent K<X115. Radiographically, the KCOf
mandibular ramus and posterior body are the most com- appears as a well-dralmscribed radiolucency with a dis-
mon locations for the KCOf (Fig. 132.5A and B). The tinct border, and many cause bony expansion or erosion
KCOf in the maxilla typically favors the posterior or of either corta. Involved teeth may become displaced, but
canine regions. root resorption rarely occurs.
2102 Section VII: Head and Neck Surgery

Histopathology and non-sun-exposed areas, frontal bossing, mandibular


The KCOT has the following distinct microscopic appear- prognathism, palmar and plantar pitting, bifid ribs, and
ance: its lining is parakeratinized, stratified squamous epi- calcification of the falx cerebri. The KCOTs associated with
thelium, which is six to eight cell layers thick; the luminal NBCCS should be treated in a similar manner as an iso-
surface is covered by a corrugated layer of parakeratin; the lated KCOf. However, increased vigilance with 6 month
basal layer is palisaded and cuboidal, with prominent, or yearly clinical follow-up and imaging (panoramic
intensely stained hyperchromatic nuclei; and a lack of rete radiograph or computerized tomography) are warranted
pegs. This flat epithelial-connective tissue interface results to allow early detection of new lesions. This is particu-
in a separation of the epithelium on histopathologic pro- larly true of children and adolescents. Once adulthood is
cessing. Exfoliated parakeratin often fills the cyst lumen reached, the formation of new KCO'IS is less problematic,
and presents during surgery as a creamy, white material. but the risk of basal cell carcinomas rises. Other than the
Daughter (satellite) epithelial islands and cysts may be skin lesions and those conditions that may be correctable,
seen in the surrounding connective tissue and may be many of the other related abnormalities do not require any
indicative of the NBCCS. surgical intervention. Genetic counseling should be recom-
mended to both the patient and family members due to
Treatment and Prognosis the condition's autosomal dominant inheritance.
The KCOT requires surgical enucleation and osseous curet-
tage, and every attempt should be made to remove it in one
Ameloblastoma
piece (18). Modified Camoy solution is a tissue fixative
that can aid in a more complete lesion removal. Treatment With the exception of the odontoma, the ameloblastoma
of the residual bony cavity may devitalize microscopic rem- is the most common odontogenic tumor (27). It arises
nants, thus decreasing the chance of recurrence (19,20). from any number of residual epithelial elements of tooth
Some clinicians have advocated marginal resection to development: reduced enamel epithelium, rests of Serres,
avoid recurrence (21). When large lesions are present, mar- rests of Malasse.z, or the basal layer of the oral mucosa. The
supialization may be performed to decompress the KCOT lesion can also develop from within a dental follicle or a
to allow for easier removal of the cyst at a later surgery dentigerous cyst (3 ). Some confusion has arisen regarding
(22,23). Secondary infection, need for high patient com- the classification schema of ameloblastomas. Most refer-
pliance, and variable results limit the use of marsupializa- ences broadly categorize ameloblastomas into one of three
tion techniques. Most recurrences occur within 5 years, but groups: unicystic, solid or multicystic, or peripheral amelo-
reports ofrecurrence as long as 10 years later have been doc- blastomas. A misuse of terms and/or a lack of understand-
umented. Thus, dose follow-up is mandatory. Recurrence ing of these same terms with their overlapping meanings
of KCOf has been speculated based on several theories: can lead to an inadequate treatment decision, increasing
incomplete removal of the cyst due to its thin friable mem- the likelihood of recurrence. One example is the term uni-
brane and adherence to adjacent tissues, residual satellite eystic ameloblastoma. Unicystic ameloblastoma generally
cysts following enucleation, and remnants of dental lamina implies being amenable to enucleation and curettage. An
not associated with the KCOT in question causing de novo invasive ameloblastoma may be unicystic, having just one
cyst formation. The orthokeratinized odontogenic cyst is cystic space. However, that does not suggest that this inva-
considered by some to be a unique cyst, and by others to sive lesion should be treated by enucleation and curettage
be a variant of the KCOT. It is much less common than the merely because it was defined, properly or not, as a unicys-
KCOT and has a much lower recurrence rate (24). Unlike tic ameloblastoma.
the KCOT, the orthokeratinized odontogenic cyst is lined
by orthokeratinized stratified squamous epithelium. There Clinical and Radiographic Features
is a prominent granular layer below a noncorrugated sur- This benign, locally aggressive neoplasm is characterized
face, and the basal layer is less prominent by a slow growth pattern and may grow to profound pro-
portions, causing gross facial deformities. It is usually
asymptomatic and does not alter sensory nerve function.
Nevoid Basal Cell Carcinoma Syndrome
The posterior mandible appears to be a preferred site. The
(Basal Cell Nevus Syndrome, Gorlin
lesion has a very wide age range with a peak occurrence in
Syndrome)
the third and fourth decades, and it has no sex predilection.
The NBCCS is an autosomal dominant inherited condi- Radiographically, the lesion can appear as a unilocular or
tion that exhibits high penetrance and variable expressiv- multilocular radiolucency with ill-defined borders, making
ity (25,26). It is a result of a mutation of the PI'CH tumor it difficult to determine the exact size of the lesion. Buccal
suppressor gene located on chromosome 9q22.3-q31. and lingual cortical expansion is common. even progress-
Patients may manifest a combination of the following ing to cortical perforation (Fig. 132.6A and B). Tooth dis-
clinical and radiographic features: multiple KCOTs of placement and root resorption can occur but infrequently.
the jaws, multiple basal cell carcinomas of both exposed A variant, the desmoplastic ameloblastoma, is commonly
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2103

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

found in the anterior muilla or mandible. It contains Peripheral Ameloblastoma


dense connective tissue, and thus has a more opaque
radiographic appearance. Another v.ui.ant, the peripheral The peripheral variant of the ameloblastoma is a soft tissue
ameloblastoma. ia u.maJ.ly pre8ent on the gingival and hu mass involving the gingiva and mucosa that often cauaes
no radiographic featurea, acept perhaps a '"cupping" of separation of involved teeth (31). If radiographs confirm
underlying alveolar bone. involvement of the underlying alveolar bone, then a block
resection containing the involved mucosa. underlying
Histopathology periosteum, alveolar bone, and teeth, is performed. A 1.0-
Ameloblastomas are histologically divme. 1hey may to 1.5-cm margin of uninvolved tissue is recommended.
ahibit areas of focal variation, so adequate sampling must An en bloc resection is not necessacy if supportive radio-
be undertaken. The ameloblastoma is unencapaulated, graphic studies confirm that sufficient bone will still exist
so it typically edtibits an infiltrative growth pattern into after a sufficient maJ:Ein of tiaaue is acised to avoid a
surrounding tissues. The basal cells in the epithelium are pathologic fracture.
columnar and hyperchromatic. The nuclei are polarized
away from the basement membrane CWreveae polarllf). Adenomatoid Odontogenic 1\lmor
Numerous histologic patterns have been noted in amelo-
bla.stoma.s; however, there is no current consensus that The Aar is a benign hamartoma of odontogenic epithe-
these di.fferent histologic patterruJ have different biologic lium charactr::rized by slow, progressive growth.
behaviors (28,29). The two most frequent patterns are the
follirular and plexiform (Fig. 132.6C and D). Clinical and Radiographic Features
The MYf typically ocCUIS in adolescent or teen females.
Treatment and Prognosis It has a strong predilection for the anterior maxilla and
The primary treatment principle for any intrabony ame- ia often associated with an unerupted canine tooth (32).
loblastoma is complete removal, regardless of the tech- Most AOI's are less than 3 em in diameter, but they can
nique, due to ita locally destructive potential and high risk approach significant size and cause pain and displace
for recurrence (30) . Enucleation and cu:rettage was once tooth roots. Radiographically, the Aar usually is a wdl-
considered the recommended treatment for the unicystic ciraunscribed unilorular radiolucency associated with an
ameloblastoma. H~, curettage of the bone adjacent impacted tooth. Some lesions may have radiographically
to an ameloblastoma is now discouraged because of the evident small foci of calcifications. Root resorption is rarely
risk of seeding foci of ameloblastoma either deeper into seen with the AUf, and is typically mild.
the bone or into adjacent tissues. Furthermore, enucle-
ation alone mould be avoided when the lesion of con- Histopathology
cern is large enough that a pathologic fracwre could The Aar is highly cellular with spindle-shaped cells in
occur. Recurrence rates between 15% and 35% have been whorls or rosettes (Fig. 132. 7). Bnameloid, dentinoid, or
reported with unicystic ameloblaatomas treated by enucle- cementum-like material can be found throughout this
ation and curettage alone. A 1.0- to 1.5-em bony margin lesion. These calcifications suggest the lesion's development
is recommended for a unicystic ameloblastoma. When an
ameloblastoma grows into or completely through the con-
nective tiaaue layer surrounding the lesion, or if it recurs,
then a more aggressive treatment is required. When resec-
tion is warranted, one uninvolved overlying anatomic
barrier margin ia advocated (Fig. 132.6E and F) (3).
A bony margin of 1.5 to 2.0 em is recommended for solid
or multicystic ameloblastoma.s. When hard and soft tis-
sue margins are negative, a cure rate of nearly 98% can
be achieved. Recurrence rates as high as 90% have been
reported when more aggressive amelobla.stomas are inad-
vertently, or inadequately. treated with curettage. A 5-year
follow-up ia required, but a 10-}al' follow-up is prudent
When metastases have been reported, the lesions were his-
tologically benign like the primary tumor. These lesions
are referred to as malignant ameloblastomas, although they
do not show cytologic features typically aasociated with
malignancy. When the primary lesion is found to con- Figure 132.7 Adenomatold odontogenic cyst. Odontogenic
epithelium forming duct-lib structures lined by columnar cells
tain cytologic features of malignancy, it is classified as an exhibiting rvverse polarity. Caldflc:at:lons, a common feature of this
ameloblastic carcinoma. lesion, are seen In the lower right portion.
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2105

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.

Treatment and P10gnosis


Cumtive treatment requires resection with a 1.0-an bony
margin and a layer of overlying soft tissue. The lesion is
unencapsulated, so infiltration into adjacent bone is com-
mon making recurrence likely if only enucleation and
curettage is undertaken. Enucleation and curettage may Figu,. 132.9 Ameloblanc fibroma. Cords of odontogenic epi-
be considered only for smalL unilocular lesions (less than thelium in a background of cellular, primitive mesenchymal tissue.
1 em in diameter), but aggressive bony curettage is rec-
ommended. The inferior alftolar nerve is not routinely
within approximately 1 year. La!ger lesions (greater than
sacri:ficed, even in l;uge lesions that require an en bloc
5 em) may require a resection based on the specific anatomic
resection, unless the ner:ve has been signi:ficandy compro-
boundaries involved. Reported :recwrenctS were likely due
mised by the lesion.
to regrowth of the wmo1;. which is not completely unusual
for embryonal-type tumOIS. A diagnosis of ameloblastic
Ameloblastic Fibroma fibrosaocoma should be considered if this lesion recurs.

1he ameloblastic :fibroma is a true neoplasm composed of RELATED JAW LESIONS


both odontogenic epithelium and ectomesenchyme. The
epithelium resembles dental lamina or ameloblastoma, Various nonodontogenic lesions of the jaws are worthy
and the mesenchyme resembles the developing dental of mention due to their biologic behavior as well as the
papilla or myxoma diagnostic and surgical challenges they pose. Some believe
that these conditions may indeed be odontogenic because
Clinical and Radiographic Features many are only found in the jaws even though they do not
The ameloblastidibroma is a tumor of young patients with reveal histological features consistent with odontogenic-
rare occurrence over the age of 40. It presents as a painless derived structures.
swelling usually in the third molar region of the mandible.
Enl;ugement of the affected jaw is the only frequent clini- Torus
cal sign. The lesion can be either a unilocular or multilocu-
A torus is a developmental ovetgrowth rather than a tumor
lar radiolucency with a sclerotic border. It often occurs over
or hamartoma and is thought to arise due to bone stress
an unerupted tooth and can displace the tooth but does
(39 ). It develops in one of two intmoral sites. When it ocaus
not cause root resorption. The ameloblastic fibroma does
on the midline of the palate. it is termed torus palatinus.
not have a pathognomonic radiographic feature and can
When tori oa:ur on the lingual aspect of the mandible, they
resemble a dentigerous cyst.
are typically bilateral and adjacent to the canine/bicuspid
region. lhese are termed tmus mandibularis or lingual tori.
Histopathology When a histologically similar lesion develops on the buccal
Microscopically, the tumor consists ofislands ofodontogenic aspect of either jaw, the lesion is refeued to as an exrutosis.
epithelium resembling the dental lamina and cap stage of
odontogenesis. This columnar epithelium is palisaded and Clinical and Radiographic Features
showa striking nuclear polarity similar to that of the amel~ The palatal torus develops after puberty. It can be found in
blutoma (Fig. 132.9). The stroma is myxoid in appeamnce, approximately 20% ofadults and has a slow growth pattern.
resembling the dental papilla, a cell-rich. myx.oid tissue. Palatal tori can either have a smooth ovoid appearance or
multiple pedunculated loculatiorua, but both presentations
Treatment and P10gnosis should have normal pink overlying mucosa. Tori can grow
The ameloblastic fibroma can be cured through enucleation to large dimensiorua, impairing speech or feeding or pro-
and curettage since it is well encapsulated. Owing to its pre- hibiting fabrication of a maxillary prosthesis (Fig. 132.10).
dilection for young patients, the bony defect will repair itself Also, large lesions are often prone to mucosal ulcemtion
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2107

osteoma). When multiple osteomas are present, one should


consider that the patient may have G4nlner syrulrome, an
autosomal dominant condition also associated with intf!sti-
nal polyposis, epidermoid cysts, and desmoid tumors of the
skin. impacted normal and supernumerary teeth, and odon-
tomas (40). 1he formation of the osteomas will usually pre-
cede other manifestations of the syndrome. If this syndrome
is suspected,. the appropriate referral should be made to rule
out intf!Stinallesions, particularly polyps, which have a high
rate of malignant transfonnation to colorectal cancei: The
fu!quency of malignant transformation is essentially 100%
in these patients as they reach older age.

Oinical and Radiographic Features


These slow-growing. asymptomatic exophytic bony masses
OCOJJ in either jaw in areas not typically affected by tori
Figure 132.10 Palmi torus. or exostoses. The mandibular angle is a common location.
The lesion is usually an incidental :finding on routine radio-
graphic evaluation and appealS as a well-circumscribed
from mastication. 'Ihese larger lesions may require surgical radiopaque mass.
excision. Mandibular tori are commonly bilateral and also
have a slow growth pattern. They too can reach sizable Histopathology
dimensions, adversely affecting speech or feeding or the Osteomas have similar microscopic features as tori or exos-
use of a lower prosthesis. toses. The periosteum can be more active in the osteoma
than the other two lesions.
Histopllthology
Thri consist of nodular masses of dense cortical, lamellar Treatment and Prognosis
bone with central trabecular bone containing few areas of Asymptomatic single lesions can be followed clinically and
fi.brofatty marrow. radiographically. Those lesions requiring biopsy are surgi-
cally excised with litde chance for recurrence.
TnN~tment and Prognosis
Tori only require removal if they intf!lfe:re with normal
function or the fabrication and placement of a prosthesis
Osteochondroma
such as a denture. An elliptical or double-Y-shaped muco- Osteochondromas are benign hamartomas that develop
sal incision is designed, and the lesion is taken down to most commonly in long bones, but also occur in the man-
the level of surrounding bone with a rotary instrument. dibular condyle or coronoid process (41). The lesion is
1he torus can also be scored in the shape of a cross with believed to be associated with proliferation of epiphyseal
a surgical drill then removed with an osteotome and cartilage into the surrounding tissues.
mallet. Care must be taken not to perforate through the
nasal floor. A surgical stent can be created preoperatively Clinical and Radiographic Features
so that the swgical site can be postoperatively protected The absence of this lesion in other portions of the mandi-
from irritation from either the tongue or fooc:b. Removal of ble, skulL or facial bones serves to confirm its development
mandibular tori requires attention to the submandibular in endochondral bone. Osteochondromas are lesions
salivary ducts when one is using an osteotome and mallet of younger patients, usually occurring in the second and
to excise these lesions. A drill can be used to make vertical third decades, and are found in twice as many males as
cuts along the inner aspect of the torus between the lesion females. The lesion is slow growing and lmown to cause
and the alveolus. An osteotome is then used to outfracture swelling and pain along with deviation of the teeth and
the torus. Then a rotary instrument is used to smooth the chin point toward the unaffected side. Radiographically, it
edgl!S of the lingual shelf prior to closure of the flap. appears as an irregular '"popcorn-like" radiopaque mass on
the medial side of the condyle or replacing the coronoid
process (Fig. 13l.11A and B).
Osteoma
Osteomas are hamartomas or reactive proliferations of bone Histopathology
and are not considered to be true tumors. 1hey are com- Osteochondromas are bony masses that have a cartilagi-
posed of dense compact bone and arise either on the sur- nous cap. Endochondral ossification is noted between the
face of bone (periosteal osteoma) or within bone (endosteal cartilage and bone (Fig. 13l.11C).
2108 Section VII: Head and Neck Surgery



'

.. •

Figure 132.11 A:. Osteochondroma. Panoramic radiograph of


a 53-yea,..old man who presented with right preauricular swell·
lng, discomfort, and left-sided shift In his occlusion. Radiograph
demonstnJtes Inhomogeneous lesion of right condyle that appears
to have replaced entlno~ normal bony archltectuno~. 1: Three·
dimensional reformattad computed tomography (CT) sam of right
side as viewed from below. Lateral and more extensive medial
extension of the lesion Is seen. C: Osteochondroma. Chondroid
matrix with foci of developing osteocytes and osteoid. D: A right
condylectomy was performed through a combined preauricular
an~ retro'!'andibular approach. A soft tissue capsule surrounding
ent1re reg1on was encountered. Specimen seen from anterior view. D

Treatment and P10gnosis Ossifying Fibroma (Cementoossifytng Fibroma)


Lesions affecting the coronoid process are managed by a
coronoidectomy with minimal removal of the attached Ossifying fi.bromu are true benign neoplums of mes-
temporalis muscle tendon. Lesions of the condyle are enchymal origin that have a strong predilection for the
tooth-bearing portion of the jaws, although they have been
treated by condylectomy with a preauricular as well u a
neck incision (Fig. 132.110). Immediate reconstruction reported in long bones (42). This slow-growing expansile
can be treatment planned with either a costochondral graft lesion can reach enormous dimensions resulting in pro-
or an alloplastic condyle. Recurrence is very rare. found facial disfigurement.
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2109

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.

Treatment and P10gnosis


Fibrous dysplasia does not require swgery unless the
lesion is disfiguring and the patient desires a more normal
and esthetic appearance or rarely if there is cranial nerve
dysfunction (44). Recontouring of the affected jaw or facial
bone is performed rather than resection. and surger:y is
usually delayed Wltil the affected bone has reached matu-
rity (Fig. 132.13E). Reports of sarcomatous transformation
have been documented, and recurrence is more likely if the
lesion is treated during an active growth period.

Central Giant Cell Lesion


The central giant cell lesion (CGCL) is a locally aggressive
Figure 132.12 Ossifying fibroma. Irregular 1rabeculae of bone benign lesion that occurs in both long bones and the jaws.
are seen throughout a aallular, fibrous connective tissue stroma. Jaw lesions may have similar histopathologic features as
2110 Section VII: Head and Neck Surgery

Figure 132.13 A:. Fibrous dysplasia. Panoramic radiograph of a


28-year-old man who presented with steadily inaeasing right man·
dibular and facial enlargement, which had been monitored over
several years' time. Biopsies established diagnosis of fibrous dys-
pla,ia. B: Coronal <XImputed tomography (CT) scan of miVIillofa-
cial bone' demonstrates frontal, mallillary, and mandibular bony
involvement. C: Three-dimensional refonnatted CT scan depicting
the gross deformity of the right mandibular lesion. D: Fibrous dys-
plasia. Curvlllnear portions of wovcm bone, without appositional
osteoblast's, In a background of <XInnc~e:tfve tissue stroma. E: Gross
specimen of the fibrous dysplasia. E
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2111

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.

Clinical and Radiographic Features Clinical and Radiographic Features


CGCLs of the jaws most often are asymptomatic and are The ABC commonly occurs in the first three decades of
not recognized until they present as a painless swelling. life. They have a predilection for the posterior portions
A more aggressive form of the CGCL can cause pain or par- of the jaws. They present as firm swellings that can be
esthesia. Clinically, the condition can appear as a bluish diffuse. Teeth may become loosened and displaced, and
mass as a result of the thinning of the overlying bone and root resorption may occur, but the teeth remain vital.
mucosa and its highly vascular nature. Most cases develop Radiographically, ABCs are expansile, often multilocular,
in the second to third decades, and women outnumber radiolucencies. The borders are usually well demarcated,
men 2: 1. The lesion favors the anterior region of the jaws, but cortical perforations with extension into the soft
particularly the mandible, often crossing the midline, and tissues do occur.
is occasionally seen bilaterally. The CGCL appears as either
a well-circumscribed radiolucency or a multilocular radio- Histopathology
lucency. Larger lesions can cause cortical displacement The large blood-filled spaces are separated by connective
(Fig. 132.14A). The CGCL can also displace teeth, but root tissue septa. Hemosiderin, bone, and osteoid can be found
resorption is not typical. within the septa. Osteoclast-type multinucleated giant
cells, similar to those found in the CGCL, are a common
Histopathology feature at the periphery of the lesion. Mitoses are com-
The associated giant cells behave much like osteoclasts. monly found, but atypical forms are not
Multinucleated giant cells varying in size and their num-
ber of nuclei are dispersed throughout a background of Treatment and Prognosis
spindled mesenchymal cells. The lesion is unencapsu- The ABC is managed by curettage, and intraoperative bleed-
lated, and fibrous tissue is also present to varying degrees ing can become a challenge. Embolization may become
(Fig. 132.14B). Areas of hemorrhage and hemosiderin necessary in select cases.
deposits are commonly found. Immunologic stud-
ies support the biologic behavior and notion that the
giant cells represent osteoclasts. Histologic features are Vascular Malformations
not predictive of its biologic behavior, and the CGCL is Vascular malformations are developmental lesions that can
identical to several other conditions affecting the jaws, affect soft tissue or bone. They do not present at birth like
including cherubism, the brown tumor of hyperparathy- hemangiomas, which are actual neoplasms. Vascular mal-
roidism, and focal areas of fibrous dysplasia. Thus, it is formations can be broadly classified as either arterial (high-
prudent to rule out these similar lesions if the clinical flow) or venous (low-flow) malformations. Arteriovenous
features suggest that the lesion may be something other fistulas may also be considered high-flow lesions.
thana CGCL.
Clinical and Radiographic Features
Treatment and Prognosis Most vascular malformations within the jaws present
Curettage is usually curative, but recurrent or larger lesions as slow-growing, asymptomatic, expansile lesions ( 49).
occasionally require resection (F"tg. 132.14C and D). The associated teeth become clinically mobile and may
Recurrence becomes an issue if the lesion is associated with even appear elevated. Thrills or bruits may be detectable
difficult removal-attachment to multiple roots or neuro- on physical examination. When the tongue is involved,
vascular structures, or large~; more vascular lesions. Several lingual veins become distended. Vascular malformations
nonsurgical therapies have been reported with some degree take on a variable radiographic appearance. Some may
of success. Intralesional steroid injections using triamcino- appear as well-circumscribed radiolucencies, whereas
lone (10 mgfmL) weekly for 6 weeks is advocated by some others may appear as more mixed radiolucent/radi-
as a first line of therapy (47). Another reported injectable opaque. Tooth roots may be resorbed with high-flow
therapy is calcitonin ( 48). The precise mechanism of action lesions. A cr scan or MRI should be obtained to aid in
of calcitonin on the giant cell tumor is unknown. but the ascertaining the extent of the lesion, and angiography is
giant cells have been shown to have calcitonin receptors, performed to assist in determining the primary vascular
and calcitonin somehow interferes with the progression of inflow and the presence of any contralateral vascular con-
the tumor. tributions.
2112 Section VII: Head and Neck Surgery

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

Histopllthology tissues. Hypotensive anesthesia can also be used to further


Thrtuous endothelium-lined, dilated vessels comprise the reduce inttaoperative bleeding. Venous malformations can
microscopic appearance of this condition. Adjacent smaller be treated with coils or inttalesional injections of scleros-
feeding vessels can also be seen in some cases. ing agents. If adequate thrombosis is achieved, then the
lesion can be curettaged.
Treatment and PIVgnosis
Any radiolucent lesion of the jaw, which might be clini- CONCLUSION
cally considered to be a vascular malformation, requires
needle aspiration prior to biopsy or sw:ge:ry to rule this Collectively, odontogenic cysts and tumors do not occur
condition out. Arterial malformations are treated with with considerable frequency. Many of these lesions are
preoperative selective embolization followed by resection amenable to sw:gical enucleation and curettage. Some of
surgery. The purpose of embolization is to limit the blood the cysts and tumors possess locally aggressive behavior
:flow to the lesion so as to minimize blood loss when the and are capable of great tissue distortion and/or destruc-
lesion is excised. A 'Valiety of materials can be used to tion. Thus, they present challenges regarding their treat-
embolize these lesions: coils, polyvinyl alcohol beads, or ment and ultimate reconstruction. Long-tf!rm follow-up
100% alcohol (SO). Overaggressive embolization must is advisable for many of these lesions, as recurrences
be avoided to prevent ischemic necrosis and sloughing of do oca:u:
Chapter 132: Odontogenic Cysts, Tumors, and Related Jaw Lesions 2113

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