Odontogenic Tumors

Classification of Odontogenic
Tumors*

I. Tumors of odontogenic epithelium

A.
Ameloblastoma
1. Malignant ameloblastoma
2. Ameloblastic carcinoma
B.
Clear cell odontogenic carcinoma
C.
Adenomatoid odontogenic tumor
D.
Calcifying epithelial odontogenic tumor
E.
Squamous odontogenic tumor
II. Mixed odontogenic tumors
A.
Ameloblastic fibroma
B.
Ameloblastic fibro-odontoma
C.
Ameloblastic fibrosarcoma
D.
Odontoameloblastoma
E.
Compound odontoma
F.
Complex odontoma
III. Tumors of odontogenic ectomesenchyme
A.
Odontogenic fibroma
B.
Granular cell odontogenic tumor
C.
Odontogenic myxoma
D.
Cementoblastoma

* From Neville, et al.

Biologic Classification of Odontogenic Tumors*

BENIGN, NO RECURRENCE
POTENTIAL
Adenomatoid odontogenic tumor
Squamous odontogenic tumor
Cementoblastoma
Periapical cementoosseous dysplasia
Odontoma
BENIGN, SOME RECURRENCE
POTENTIAL
Cystic ameloblastoma (unicystic)
Calcifying epithelial odontogenic
tumor
Central odontogenic fibroma
Florid cementoosseous dysplasia
Ameloblastic fibroma and
fibroodontoma
*From Regezi, et al.

BENIGN AGGRESSIVE
Ameloblastoma
Clear cell odontogenic tumor (some
consider this a carcinoma)
Odontogenic ghost cell tumor
(COC, solid type)
Odontogenic myxoma
Odontoameloblastoma

MALIGNANT
Malignant ameloblastoma
Ameloblastic carcinoma
Primary intraosseous carcinoma
Odontogenic ghost cell carcinoma
Ameloblastic fibrosarcoma

Tumors of Odontogenic
Epithelium

Ameloblastoma

The ameloblastoma is the most common

clinically significant (not the most common)
odontogenic tumor.
It may develop from cell rests of the enamel
organ; from the developing enamel organ; from
the lining of odontogenic cysts or from the basal
cells of the oral mucosa.

Ameloblastoma

It is typically slow-growing, locally invasive and

runs a benign course.
H.G.B. Robinson described it as being a benign
tumor that is usually unicentric, non-functional,
intermittent in growth, anatomically benign and
clinically persistent.

Ameloblastoma

Ameloblastomas occur in 3 different clinicoradiographic situations requiring different

therapeutic considerations and having different
prognoses.
Conventional Solid/Multicystic (86 % of all cases)
Unicystic (13 % of all cases)
Peripheral or Extraosseous (1 % of all cases)

Solid or Multicystic Ameloblastoma:

Clinical Features

Patient Age: Approximately equal frequency from the

third through the seventh decades.
Sex Predilection: Approximately equal.
Location: 80 % in mandible; 70 % in posterior regions.
Radiographic Appearance: Radiolucent lesion which is
usually well-circumscribed; it may be unilocular or
multilocular (soap-bubble, honeycomb); occasionally an
ameloblastoma will be ill-defined with a ragged border.

Solid or Multicystic Ameloblastoma:

Histologic Features

There are several microscopic subtypes but

these generally have little bearing on the
behavior of the tumor.
The follicular and plexiform types are the most
common.
The follicular type is composed of islands of
epithelium which resemble the enamel organ in
a mature fibrous connective tissue stoma.

Solid or Multicystic Ameloblastoma:

Histologic Features

The plexiform type is composed of long,

anastomosing cords or larger sheets of
odontogenic epithelium. Its stroma tends to be
loose and more vascular.
The acanthomatous type shows evidence of
extensive squamous metaplasia with keratin
formation in the island of odontogenic
epithelium.

Solid or Multicystic Ameloblastoma:

Histologic Features

In the granular cell type there is transformation

of groups of epithelial cells to granular cells; the
nature of the granular change is unknown. This
type is more common in young patients and has
been shown to be clinically aggressive.

Solid or Multicystic Ameloblastoma:

Histologic Features

The desmoplastic form is composed of

islands/cords of odontogenic epithelium in a
very dense collagenous stroma. It has a
predilection for the anterior maxilla and because
of the dense connective tissue may appear as a
radiolucent-radiopaque lesion.
The basaloid type is the least common and is
composed of uniform basaloid cells with no
stellate reticulum.

Solid or Multicystic Ameloblastoma:

Additional Features

In some studies solid/multicystic

ameloblastomas are reported to be more
common in Blacks.
While lesions are generally asymptomatic,
ameloblastomas may cause paresthesia, pain
particularly if infected and they can erode the
cortical palates.

Solid or Multicystic Ameloblastoma:

Treatment

Treatments have ranged from simple

enucleation and curettage to en bloc resection.
Marginal resection is the most widely used
method of treatment with the least recurrences
reported (up to 15 %).
Most surgeons advocate a margin of at least 1.0
cm beyond the radiographic limits of the tumor
as the tumor often extends beyond the apparent
radiologic/clinical margins.

Solid or Multicystic Ameloblastoma:

Prognosis

Treatment with curettage has resulted in

recurrence rates ranging from 55-90 %.
Treatment with marginal resection has resulted
in approximately a 15 % recurrence rate.
Ameloblastomas of this type arising in the
maxilla are particularly dangerous as it is often
difficult in getting adequate margins.
Rarely is an ameloblastoma life threatening.

Unicystic Ameloblastoma: Clinical

Features

Patient Age: The patients are younger than

those with the solid/multicystic form. 50% are
diagnosed during the second decade of life.
Sex Predilection: ? Same as for the solid??
Location: 90 % occur in the mandible usually in
the posterior region.
Radiographic Appearance: Typically appears as
a RL around the crown of an unerupted tooth
(most commonly a mandibular third molar).

Unicystic Ameloblastoma:
Histologic Features

Three histopathologic variants are recognized:

Luminal: the tumor is confined to the luminal
surface of the cyst.
Intraluminal/plexiform: the tumor projects from
the cystic lining; sometimes resembles the plexiform
type of solid/multicystic ameloblastoma.
Mural: the tumor infiltrates the fibrous cystic wall.

Unicystic Ameloblastoma:
Treatment and Prognosis

Enucleation of the cyst is probably adequate for

the luminal and intraluminal/plexiform types.
Treatment of the mural type is controversial
with some surgeons believing that local
resection is best.
10-20 % recurrence after enucleation and
curettage with all unicystic ameloblastomas.

Peripheral Ameloblastoma

These tumors are extraosseous and therefore

occupy the lamina propria underneath the
surface epithelium but outside of the bone.
Histologically, these lesions have the same
features as the intraosseous forms of the tumor.

Peripheral Ameloblastoma: Clinical

Features

Patient Age: Wide age range but most occur

during middle-age.
Gender Predilection: This is not known.
Location: Posterior gingival/alveolar mucosa is
involved most frequently. There is a slight
predilection for the mandible. The buccal
mucosa has been the site in a few reported cases.

Peripheral Ameloblastoma:
Radiographic & Histologic Features

Radiographic Appearance: Although not in

bone, a few cases have shown superficial erosion
of the alvelolar bone.
Histologic Appearance: Islands of ameloblastic
epithelium are observed in the lamina propria;
plexiform and follicular patterns are the most
common; in 50 % of the cases the tumor
connects with the basal cell layer of the surface
epithelium.

Peripheral Ameloblastoma:
Treatment and Prognosis

Unlike its intraosseous counterpart, this tumor

has an innocuous clinical behavior.
Patients respond well to local surgical excision.
Some reports indicate a 25 % recurrence rate but
in these cases as second surgical procedure
results in cure.
There has been a rare malignant change
reported.

Malignant Ameloblastoma and

Ameloblastic Carcinoma

Less than 1 % of the ameloblastomas show malignant

behavior with the development of metastases.
Malignant ameloblastoma is a tumor that shows
histologic features of the typical (benign)
ameloblastoma in both the primary and secondary
deposits.
Ameloblastic carcinoma is a tumor that shows
cytologic features of malignancy in the primary tumor,
in recurrence and any metastases.

Malignant Ameloblastoma &

Ameloblastic Carcinoma: Clinical

Patients range in age from 4-75 with a mean of

30 years.
Metastasis has occurred from 1-30 years after
the initial treatment.
Metastases most often occur in the lungs and in
the case of malignant ameloblastoma raises the
question of aspiration during surgery. Spread
has also occurred to the cervical lymph nodes
and to vertebrae and viscera.

Malignant Ameloblastoma &

Ameloblastic Carcinoma: X-Ray

With the malignant ameloblastoma, the

appearance is similar to the typical
solid/multicystic ameloblastoma.
The ameloblastic carcinoma is often more
aggressive with the lesion appearing as an illdefined radiolucency with cortical destruction.

Malignant Ameloblastoma &

Ameloblastic Carcinoma: Histology

With the malignant ameloblastoma, both the

primary and metastases show no microscopic
features that differ from those of the typical
solid/multicystic ameloblastoma.
The ameloblastic carcinoma shows cytological
features of malignancy in addition to a pattern
of an ameloblastoma.

Malignant Ameloblastoma &

Ameloblastic Carcinoma: Treatment
& Prognosis

Long-term follow-up does not permit accurate

assumptions to be made but prognosis appears
to be poor.
Approximately 50 % of the patients with
documented metastases and long-term followup have died as the result of their disease.

Adenomatoid Odontogenic Tumor

(AOT)

Formerly called an adenoameloblastoma, a

somewhat deceptive term that should be
discarded, the AOT represents about 3-7 % of
all odontogenic tumors.
This epithelial tumor has an inductive effect on
the odontogenic ectomesenchyme with
dentinoid frequently being produced.

AOT: Clinical Features

Patient Age: The peak age is in the second

decade with a mean around 17 years.
Gender Predilection: Females, 2:1.
Location: Sixty-five percent of the AOTs occur
in the maxilla with 65 % occurring in the canine
region. Seventy-five percent of the cases are
associated with the crown of an unerupted
tooth. On rare occasion the lesion is
extraosseous.

AOT: Radiographic and Additional

Features

AOTs typically appear as pericoronal radiolucencies,

which may have radiopaque material (snowflake
calcifications) within the lucency.
These lesions are frequently asymptomatic and
therefore are discovered upon routine radiographic
examination. AOTs may also block the eruption of a
permanent tooth and be discovered when radiographs
are taken to search for the unerupted tooth.

AOT: Histologic Features

The lesion is usually surrounded by a thick, fibrous

capsule.
The tumor is composed of spindle-shaped epithelial
cells that form sheets, strands or whorled masses with
little connective tissue.
The epithelial cells may form rosette-like structures,
tubular or duct-like structures may be prominent or
absent.
Calcifications may be observed in the tumor mass.

AOT: Treatment and Prognosis

Enucleation is the treatment of choice as the

tumor is easily removed from the bone.
AOTs seldom recur.

Calcifying Epithelial Odontogenic

Tumor (CEOT; Pindborg Tumor)

Pindborg tumor accounts for < 1 % of all

odontogenic tumors.
It is clearly of odontogenic origin but its
histogenesis is uncertain.
The tumor cells are said to resemble cells of the
stratum intermedium.

CEOT: Clinical Features

Patient Age: Patients ages range from the

second to the tenth decades with a mean around
40 years.
Gender Predilection: There is no reported sex
predilection.
Location: 75 % of the CEOTs occur in the
mandible with most occurring in the posterior
region. A rare peripheral CEOT does occur.

CEOT: Radiographic Features

CEOTs occur as radiolucent lesions

with/without opaque foci.
They are usually well-circumscribed and may be
unilocular or multilocular.
Slightly over 50 % of the CEOTs are associated
with an unerupted tooth.

CEOT: Histologic Features

This lesion is typically composed of islands, sheets or

strands of polyhedral epithelial cells in a fibrous stroma.
Areas of amorphous, eosinophilic, hyalinized
extracellular material may be scattered throughout.
Cells outlines are distinct and intercellular bridges may
be seen.
Nuclei show considerable variation with giant nuclei
and pleomorphism observed.
Calcifications may be noted as well as amyloid-like
material. Liesegang rings also may be present.

CEOT: Additional Features,

Treatment and Prognosis

Bony lesions most commonly present as painless, slowgrowing swellings.

Peripheral lesions typically appear as non-specific
sessile gingival masses.
Conservative local resection is the treatment of choice
as these lesions are typically less aggressive than the
ameloblastoma.
With this treatment the recurrence rate is approximately
15 % and the overall prognosis is good.

Squamous Odontogenic Tumor

(SOT): Clinical Features

Patient Age: Second through the seventh

decades (mean 40 years).
Gender Predilection: None
Location: SOTs occur with about equal
frequency in maxilla and mandible. They are
more common in the anterior regions of the
jaws than in the posterior. The lesions occur in
the alveolar process.

SOT: Radiographic and Histologic

Features

SOTs appear as non-specific radiolucent lesions.

They may be well-circumscribed or ill-defined.
They often appear triangular in shape and lateral
to the tooth root.
Histologically, they appear as islands of blandappearing squamous epithelium in a mature
fibrous connective tissue stroma. The
peripheral cells do not show the characteristic
polarization seen in the ameloblastoma.

SOT: Additional Features,

Treatment and Prognosis

SOTs often present as painless gingival swellings

associated with tooth mobility. Approximately
25 % are asymptomatic.
Conservative local excision or curettage appears
to be effective treatment and there have only be
a few recurrences reported.

Mixed Odontogenic
Tumors
This group of tumors is composed of
proliferating odontogenic epithelium in a
cellular ectomesenchyme resembling the
dental papilla.

Ameloblastic Fibroma: Clinical

Features

This true mixed odontogenic tumor is more

common in patients in the first and second
decades of life with a mean of 14 years.
It is slightly more common in males than
females.
Approximately 70 % of the ameloblastic
fibromas occur in the posterior mandible.

Ameloblastic Fibroma:
Radiographic Features

Generally, these lesions appear as either a

unilocular or multilocular radiolucency.
They tend to be well-defined and may have a
sclerotic border.
Approximately, 50 % are associated with an
unerupted tooth.

Ameloblastic Fibroma: Histologic

Features

The tumor is composed of a cell-rich

mesenchymal tissue resembling the primitive
dental papilla admixed with proliferating
odontogenic epithelium.

Ameloblastic Fibroma: Additional

Features, Treatment and Prognosis

The tumor is often encapsulated with small

tumors usually being asymptomatic. Larger
tumors produce swelling, which can expand the
cortex and be quite pronounced.
Most ameloblastic fibromas are treated by
conservative surgical excision; however, a 20 %
recurrence rate has led some surgeons to
recommend a more aggressive approach.

Ameloblastic Fibro-odontoma

This lesion is defined as a tumor with general

features of an ameloblastic fibroma but
containing enamel and dentin.
Some investigators believe that this entity is but
a stage in the development of an odontoma;
however, most agree that progressive destructive
tumors are true neoplasms.

Ameloblastic Fibro-odontoma:
Clinical and Radiographic Features

Patient Age: Most common in the 5-12 year age range

with a mean of 10 years.
Gender Predilection: None.
Location: It is more common in the premolar/molar
regions of both jaws.
Radiographic Features: Usually appears as a welldefined unilocular or rarely multilocular radiolucency
with variable amounts of calcified material which is
radiopaque. Therefore, it may appear as a mixed,
radiolucent-radiopaque lesion.

Ameloblastic Fibro-odontoma:
Histologic Features

The soft tissue component is identical to the

ameloblastic fibroma. The calcified portion
consists of foci of enamel and dentin matrix
formation in close relationship to the epithelial
structures.

Ameloblastic Fibro-odontoma:
Treatment and Prognosis

The ameloblastic fibro-odontoma is usually

treated by conservative curettage with the lesion
separating easily from the surrounding bone.
Prognosis is excellent and recurrence is unusual.

Ameloblastic Fibrosarcoma

This lesion is considered the malignant

counterpart of the ameloblastic fibroma in
which the mesenchymal portion shows features
of malignancy.
The ameloblastic fibrosarcoma may arise de
novo or there may be a malignant
transformation of an ameloblastic fibroma.

Ameloblastic Fibrosarcoma: Clinical

and Radiographic Features

Patient Age: The mean age for patients with

ameloblastic fibrosarcoma is 26 years.
Gender Predilection: Males 2:1.
Location: 75 % have occurred in the mandible.
Radiographic Features: Appear as an ill-defined,
destructive, radiolucent lesion.

Ameloblastic Fibrosarcoma:
Histologic Features

The epithelial component of this tumor appears

histologically benign.
The mesenchymal portion is highly cellular. The
cells are hyperchromatic and quite pleomorphic.
Mitoses are usually prominent.

Ameloblastic Fibrosarcoma:
Additional Features, Treatment &
Prognosis

Pain and swelling are typically associated with

this tumor. Rapid clinical growth is another
common feature.
Radical surgical excision is the treatment of
choice.
The long-term prognosis is difficult to ascertain
because of the small number of reported cases.

Odontoma

The odontoma is the most common odontogenic

tumor.
It is not a true neoplasm but rather is considered to be
a developmental anomaly (hamartoma).
Two types of odontomas are recognized:

Compound: this type of odontoma is composed of multiple

small tooth-like structures.
Complex: this lesion is composed of a conglomerate mass of
enamel and dentin, which bears no anatomic resemblance to
a tooth.

Odontoma: Clinical Features

Patient Age: Most cases are recognized during

the second decade of life with a mean of 14
years.
Gender Predilection: Approximately equal.
Location: Somewhat more common in the
maxilla. The compound type is more often in
the anterior maxilla while the complex type
occurs more often in the posterior regions of
either jaw.

Odontoma: Radiographic Features

Early lesions are radiolucent with smooth, welldefined contours.

Later a well-defined radiopaque appearance
develops.
The compound type shows apparent tooth
shapes while the complex type appears as a
uniform opaque mass with no apparent tooth
shapes present.

Odontoma: Additional Features

Most odontomas are small and do not exceed the size

of a normal tooth in the region.
However, large ones do occur and these may cause
expansion of the jaw.
Most odontomas are asymptomatic and as a result are
discovered upon routine radiographic examination.
Odontomas may block the eruption of a permanent
tooth and in these cases are often discovered when
searching for the missing tooth radiographically.

Odontoma: Histologic Features

The compound odontoma is composed of

enamel, dentin and cementum arrange in
recognizable tooth forms; some enamel matrix
may be retained in immature and
hypomineralized specimens.
The complex odontoma is composed of enamel,
dentin and cementum but these tissues are
arranged in a random manner that bears no
morphological resemblance to a tooth.

Odontoma: Treatment and

Prognosis

Odontomas are treated by simple local excision

and the prognosis is excellent.

Tumors of Odontogenic
Ectomesenchyme

(Central) Odontogenic Fibroma:

Clinical Features

Fewer than 50 cases have been reported in the English

literature.
Patient Age: Patients have ranged in age from 9-80
years old with a mean of 40 years.
Gender Predilection: Females, 7.4:1 in one study.
Location: Sixty percent occur in the maxilla where
most are located anterior to the first molar. When in
the mandible, approximately 50 % occur in the
posterior jaw.

Odontogenic Fibroma:
Radiographic Appearance

The odontogenic fibroma usually appears as a

well-defined, unilocular radiolucency. It is often
associated with the apical area of an erupted
tooth.
Larger lesions are often multilocular.
Many odontogenic fibromas have sclerotic
borders.
Root resorption is common.

Odontogenic Fibroma: Additional

Features

Small odontogenic fibromas are usually

asymptomatic.
The larger lesions may be associated with
localized bony expansion of the jaw or with the
loosening of adjacent teeth.

Odontogenic Fibroma: Histologic

Features

Some authors have described two separate types of

odontogenic fibromas.
The simple odontogenic fibroma is composed of
stellate fibroblasts arranged in a whorled pattern with
fine collagen fibrils and a lot of ground substance.
Foci of odontogenic epithelium may or may not be
present.
Occasionally, foci of dystrophic calcification may be
present.

Odontogenic Fibroma: Histologic

Features

The WHO type odontogenic fibroma appears

as a fairly cellular fibrous connective tissue with
collagen fibers arranged in interlacing bundles.
Odontogenic epithelium in the form of long
strands or isolated nests is present throughout
the lesion.
Calcifications composed of cementoid and/or
dentinoid may be present.

Odontogenic Fibroma: Treatment

and Prognosis

The odontogenic fibroma is usually treated by

enucleation and curettage.
There have been few recurrences, this the
prognosis is good.

Odontogenic Myxoma: Clinical and

Radiographic Features

Patient Age: 10-50 years with a mean around 30

years.
Gender Predilection: Reported to be about
equal.
Location: May occur in any area of the jaws but
more common in the mandible.
Radiographic Appearance: Radiolucent lesion
often with a multilocular appearance. The
borders may be indistinct.

Odontogenic Myxoma: Histologic

Features

The tumor is composed of loosely arranged

stellate, spindle-shaped and round cells in an
abundant, loose myxoid stroma with few
collagen bundles.
Epithelial cells are not required for diagnosis.
The odontogenic myxoma may be confused
with a chrondromyxoid fibroma or with myxoid
change in an enlarged dental follicle or papilla.

Odontogenic Myxoma: Treatment

and Prognosis

Small odontogenic myxomas are treated by

curettage, while larger lesions may require
surgical resection.
Odontogenic myxomas are not encapsulated and
tend to infiltrate adjacent tissues.
Recurrence rates of up to 25 % are reported.
Overall, the prognosis is good for most
odontogenic myxomas.