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Odontogenic tumors

DR.Islam Al-obaidy
(A) Epithelial Odontogenic Tumors
Benign tumors:
1. Ameloblastoma
a. Conventional ameloblastoma
b. Peripheral ameloblastoma
c. Unicystic ameloblastoma
2. Adenomatoid odontogenic tumor
3. Calcifying epithelial odontogenic tumor (Pindborg tumor)
Malignant tumors:
1. Ameloblastic carcinoma
2. Primary intra-osseous carcinoma
(B)Ectomesenchymal Odontogenic Tumors
Benign tumors:
1. Odontogenic myxoma
2. Central Odontogenic fibroma (Central and peripheral)
3. Cementoblastoma (true cementoma)
C)Mixed odontogenic Tumors
Benign tumors:
1. Ameloblastic fibroma
2. Odontoma (compound, complex)
Malignant tumors:
1. Ameloblastic fibrosarcoma

***Cemento-Osseous Dysplasias
1. Focal cemento-osseous dysplasia
2. Periapical cemento-osseous dysplasia
3. Florid cemento-osseous dysplasia
Odontogenic lesions
• lesions derived from the tooth producing apparatus
(A) Epithelial Odontogenic Tumors
Benign tumors:
1. Ameloblastoma
a. Conventional ameloblastoma
b. Peripheral ameloblastoma
c. Unicystic ameloblastoma
2. Adenomatoid odontogenic tumor
3. Calcifying epithelial odontogenic tumor (Pindborg tumor)
Malignant tumors:
1. Ameloblastic carcinoma
2. Primary intra-osseous carcinoma
Benign tumors
1- Ameloblastoma
• Ameloblastoma is a benign locally invasive
odontogenic neoplasm of epithelial origin, which
does not metastasize
• It is progressively destructive and can reach a huge
size if left untreated.
• It is rare and only accounts for about 1 per cent of all
oral tumors
• can occur in children or the elderly
• Third molar area of either jaws {mandible (75%) -
maxilla (25%)}.
Types:
A. Conventional solid or multicystic
ameloblastoma (75% of all cases)
B. Unicystic ameloblastoma (13% of all cases).
C. Peripheral (extraosseous) ameloblastoma (about
1 % of all cases).
A. Conventional solid or multicystic
Ameloblastoma
Clinical features:
• Age: wide range, rare in children
• Site: Mandible (molar-ascending ramus)> Maxilla (posterior regions)
• Initially; asymptomatic, detected only during radiographic
examination.
• Later; painless swelling or expansion of the jaw, patient notices
gradually increasing facial asymmetry.
• If untreated, then the lesion may grow slowly to massive swelling.
• Pain or paresthesia are uncommon; but might occur if the lesion is
pressing upon a nerve.
Diagnosis:
a. Incisional biopsy, which must be kept in 10% formalin to avoid
autolysis of the specimen.
b. CT scan helps to determine areas of possible tumor extension.
• Radiographic features:
1. Multilocular radiolucent lesions having a “soap
bubble” appearance (when the radiolucent
loculations are large) or “honeycombed” (when
the loculations are small).
2. Resorption of the roots of teeth adjacent to the
tumor is common.
3. In many cases an unerupted tooth, most often a
mandibular third molar, is associated with the
radiolucent defect.

4. One form of ameloblastoma that does not have


these radiographic characteristic features is the
desmoplastic ameloblastoma. The desmoplastic
ameloblastoma has a marked predilection to
occur in the anterior regions of both jaws equally.
It shows a mixed radiolucent and radiopaque
appearance This mixed radiographic appearance
is due to osseous metaplasia within the dense
fibrous septa that characterize this lesion.
Histopathology:
➢ There are many histologic subtypes of solid and multicystic ameloblastomas.
➢ Biologic behavior of ameloblastoma is not affected by these variations.
➢ Uncapsulated, infiltrating neoplasms.

➢ There are two main patterns:


1-Follicular pattern
a. Cystic degeneration
b. Acanthomatous ameloblastoma
c. Basal cell ameloblastoma
d. Granular cell ameloblastoma
e. Desmoplastic ameloblastoma
2- plexiform pattern
1- Follicular Pattern
Epithelial islands mimic the enamel organ.
At the periphery are columnar cells, resembling
pre-ameloblasts, in which the nuclei are
polarized away from the basement membrane
and toward the center of the island (reversed
polarity).
Within the center of the island are loosely
arranged cells resembling the stellate reticulum.
Follicular ameloblastoma shows variable
histopathological subclassification, however the
biologic behavior of ameloblastoma is not
affected by these variations.
Within the stellate reticulum, the following
changes may be observed:
a. Cystic degeneration:
• Conventional ameloblastoma has a remarkable tendency to undergo
cystic change.
• In long standing tumors, extreme cystification can make the tumor
hollow.
b. Acanthomatous ameloblastoma:
• Where the stellate reticulum like cells undergo squamous metaplasia.
• Squamous metaplasia is the benign change (metaplasia) of a certain type
of epithelial cells (the stellate reticulum cells here) to a squamous
morphology.
• When this process is extensive, it is termed acanthomatous
ameloblastoma.
• Less frequently keratinization may be seen.
c. Basal cell ameloblastoma:
• The basal cell variant of ameloblastoma is the least common type.
• Composed of nests of uniform basaloid cells.
• Histopathologically are very similar to basal cell carcinoma.
• No stellate reticulum is present in the central portions of the nests
d. Granular cell ameloblastoma:
- Occasionally, the cells within the
stellate reticulum are large and contain
granular, eosinophilic cytoplasm.
- Ultra structurally and immunohistochemically,
the granules represent lysosomes.
e. Desmoplastic ameloblastoma:
• Small islands and cords of odontogenic
epithelium in a densely collagenized stroma.
(desmoplastic) tissue.
It may show osseous metaplasia in some areas.
2- Plexiform Pattern

- The second major pattern of ameloblastoma is the


plexiform pattern.
- The epithelium proliferates in a network of long
anastomosing strands or cords which are lined by
ameloblast like cells and central stellate reticulum
like cells
- Nuclei are polarized away from the basement
membrane, toward the central stellate reticulum
(reversed polarity).
- Vasodilatation of blood vessels occurs as they lose
their support after stromal degeneration; vessels
might rupture with extravasation of the red blood
cells.
- This variant is given the name “haemangio-
plexiform ameloblastoma”
Treatment:
➢ Marginal resection using 1.0- to 1.5-cm bony margins and anatomic barrier
margins.
➢ High recurrence is up to 15%; this is because the conventional ameloblastoma
tends to infiltrate between intact cancellous bone trabeculae at the periphery of
the lesion before bone resorption becomes radiographically evident. Therefore,
the actual margin of the tumor extends beyond its apparent radiographic or
clinical margin.
➢ Follow up should be at least 25 years, but lifelong follow up should be
considered
B- Unicystic ameloblastoma
Clinical Presentation:
➢ The unicystic ameloblastoma is an ameloblastoma that has a single cystic cavity.
➢ It may originate de novo as a neoplasm or as a result of neoplastic transformation of
the lining of dentigerous cyst
➢ Age: Young age, second decade
➢ Site: more common in mandible (posteriorly)
➢ Appearance: asymptomatic, although large lesions
may cause a painless swelling of the jaws.
Radiographic features:
➢ Unilocular circumscribed radiolucency that surrounds a crown of an unerupted tooth
resembling dentigerous cyst.
Types:
A* Luminal ameloblastoma
B* Intraluminal ameloblastoma
C* Mural ameloblastoma (intramural)
Histopathology
➢ Three subtypes depending upon the location and arrangement of the
epithelium.
a. Luminal ameloblastoma:
In which the neoplastic epithelium is confined to the luminal surface of
the cyst.
- The lesion consists of a simple unilocular cyst with a fibrous wall and
lined partially or completely by multiple layers of ameloblastic
epithelium.
b. Intraluminal ameloblastoma:
- One or more nodules of ameloblastoma project from the cystic lining
into the lumen of the cyst.
- The nodules may be small or large enough to fill the cyst lumen .
- This often resembles the pattern of a plexiform ameloblastoma and
hence the name plexiform unicystic ameloblastomas.

c. Mural ameloblastoma (Microinvasive ameloblastomas)


- The fibrous wall of the cyst is infiltrated by typical islands of
ameloblastic cells in follicular or plexiform pattern.
- This entity represents a change in biologic behavior from unicystic
ameloblastomas to a more invasive tumor conventional ameloblastoma.
Management:
• They are usually treated as cysts by enucleation and the patient should
be kept under long-term follow-up.
• local resection of the area is indicated as a prophylactic measure if the
tumor extends into the fibrous wall (Mural ameloblastoma)
C- Peripheral Ameloblastoma
❖ Uncommon and accounts for about 1% to 4% of all ameloblastomas.
❖ This tumor arises from:
a. rests of dental lamina beneath the oral mucosa (rests of Serres)
b. or from the basal epithelial cells of the surface epithelium
Clinical Feature:
• It will present as a firm single or polyploid mass arising exophytically from the gingiva. It
does not invade bone.
• Painless, non-ulcerated and most lesions are clinically considered to represent a fibroma or
pyogenic granuloma.
Histopathology:
❖ Histopathologically, these lesions have the same features as the intraosseous form of the
tumor.
❖ They may develop follicular, plexiform, and basilar patterns.
Radiographically: It is negative in X-ray since it is a soft tissue lesion
Treatment:
❖ Peripheral ameloblastomas are managed by local soft tissue excision with 2- to 3-mm
margins.
❖ Recurrence is not seen.
2- Adenomatoid odontogenic tumor
• It is a benign odontogenic tumor which is composed of odontogenic
epithelium with “duct like structures” and with varying degrees of
inductive changes in the connective tissue.
• It is best classified as a hamartoma rather than a true neoplasm because
of the slow growth and circumscription of the lesion.
Clinical features:
ᴥ Age: two-thirds of all cases are diagnosed when patients
are 10 to 19 years of age.
This tumor is definitely uncommon in a patient older than
age 30.
ᴥ Sex: 2/3 of the cases occur in females.
ᴥ Size: Relatively small. They seldom exceed 3 cm in
greatest diameter
ᴥ Location: It has a striking tendency to occur in the
anterior portions of the jaws and is found twice as often in
the maxilla as in the mandible.
ᴥ Could be associated with unerupted tooth (mostly
canine)
ᴥ It is frequently asymptomatic and discovered during
routine radiographic examination or when determining
why a tooth has not erupted.
ᴥ Larger lesions cause a painless expansion of the bone.
ᴥ Peripheral (extraosseous) forms of the tumor are
encountered but are rare.
ᴥ They appear as small, sessile masses on gingiva
resembling gingival fibrous lesions.
Radiographic features:
ᴥ Appears as a circumscribed unilocular
radiolucency that involves the crown of an
unerupted tooth, most often a canine.
(follicular type)
ᴥ The lesion may appear completely
radiolucent; often, however, it contains fine
(snowflake) calcifications. This feature may be
helpful in differentiating the adenomatoid
odontogenic tumor from a dentigerous cyst .
ᴥ Less often, the unilocular radiolucency
might not be related to an unerupted tooth but
located between the roots of erupted teeth
(extrafollicular type)
Histopathological Features:
1. Thick, fibrous capsule
2. Small foci of calcification.
3. Dentinoid

Treatment and Prognosis:


▪ The adenomatoid odontogenic tumor is
completely benign; because of its capsule,
it enucleates easily from the bone.
3- Calcifying epithelial odontogenic tumor
(Pindborg tumor)
It is an uncommon benign locally infiltrative epithelial neoplasm of odontogenic
origin.
Its invasiveness is less compared to the ameloblastoma.
Clinical Features:
➢ Site: more common in posterior mandibular area.
➢ Age: between 30 and 50 years (Mean age 40 years)
➢ Location: Two-thirds in the mandible, most often in the posterior areas
➢ Appears as a painless, slow-growing swelling.
➢ A few cases of peripheral (extraosseous) Pindborg tumor have been reported.
These appear as nonspecific, sessile gingival masses, most often on the anterior
gingiva.
Radiographic Features:
➢ Unilocular or multilocular
radiolucency containing
radiopaque masses of varying
size and density.

➢ May be associated with a


crown of unerupted tooth.

➢ Sometimes radiopacities are


seen reflecting the characteristic
calcified structure in this tumor.
Histopathological Features:
1.Uncapsulated
2. It is formed of sheets or strands of
epithelial cells lying in fibrous tissue.

Treatment and Prognosis:


- Conservative local resection including
a narrow rim of surrounding bone.
- The overall prognosis is good.
Malignant tumors:
1. Ameloblastic carcinoma
2. Primary intra-osseous carcinoma
Metastasizing Ameloblastoma:
• It is an ameloblastoma that metastasizes despite its benign histological features
• The metastases are usually in the lung followed by lymph nodes and bone.
Clinical Features :
There are no specific features predicting metastasis .
Histopathological feature :
Both the primary tumor and the ‘metastases’ look histologically identical to conventional benign ameloblastoma

Ameloblastic carcinoma:
• It is an ameloblastoma that has the cytologic features of malignancy in the primary tumor, in a recurrence, or in
any metastatic deposit.
Histopathological Features:
The tumor shows the microscopic pattern of ameloblastoma in addition to cytologic features of malignancy.
Radiographic Features:
Ameloblastic carcinomas are often more aggressive lesions, with ill-defined margins and cortical destruction.
• Ameloblastic carcinoma is not required to show metastasis to confirm the
diagnosis.
• While a metastasizing ameloblastoma, in contrast, must show metastasis to
be a confirmed diagnosis.

• Primary intra-osseous carcinoma is a carcinoma arising in the jaws with no


resemblance to a specific odontogenic tumor.
• It arises from odontogenic epithelium which undergoes squamous
metaplasia, or in odontogenic cysts [radicular/residual cysts are the most
common precursors followed by dentigerous cysts and odontogenic
keratocysts]
• Metastatic carcinoma must be excluded to be diagnosed as primary intra-
osseous carcinoma.
(B)Ectomesenchymal Odontogenic Tumors
Benign tumors:
1. Odontogenic myxoma
2. Central Odontogenic fibroma (Central and peripheral)
3. Cementoblastoma (true cementoma)
1- Odontogenic Myxoma
It is a benign odontogenic neoplasm that mimics microscopically the
dental pulp or dental follicle.
It is the 3rd frequent odontogenic tumor after odontoma and
ameloblastoma.

Clinical Features:
➢ Age: Wide age range 1-73
➢ Site: mandible more common than maxilla
➢ Smaller lesions may be asymptomatic and are discovered only
during radiographic examination.
➢ Larger lesions are often associated with a painless expansion of the
involved bone clinical growth of the tumor may be rapid.

Radiographic Features:
➢ Multilocular radiolucency in the form of “soap bubble” or
“honeycomb” appearance, that may displace or cause resorption of
teeth in the area of the tumor.
➢ It may be associated with an unerupted tooth.
Histopathological Features:
• Non-capsulated loosely textured tissue containing
large stellate or fusiform cells

Treatment:
• Curative treatment is accomplished by resection
with 1.0- to 1.5-cm bony margins [because
myxoma permeates medullary spaces of bone].
• Recurrence (recurrence rate 25%) is expected with
enucleation and curettage
2- Central Odontogenic Fibroma
Origin:
* It is a very rare proliferation of mature odontogenic mesenchyme.
• It may arise from the true odontogenic mesenchyme of the dental papilla, like the odontogenic myxoma, but it
differs from the myxoma by the maturity of the mesenchyme and its limited growth potential and invasive
capability.

Clinical Features:
• It has 2 clinical variants: intraosseous (or central) and extraosseous (or peripheral)
• Small tumors are asymptomatic.
• Larger tumors may show pain bony expansion and loosening of teeth.

Radiographic Features:
- A unilocular or multilocular radiolucent lesion with well-demarcated borders.
- May displace tooth roots or resorb portions of them.
Histopathology:
- It consists of spindle-shaped fibroblasts and bundles of whorled collagen fibers.
- Some lesions contain rests of odontogenic epithelium

Treatment:
▪ Enucleation and curettage. ▪ Recurrence is uncommon
3- Cementoblastoma (true cementoma)
• Rare benign odontogenic tumor that is intimately associated with roots of teeth. It is characterized by formation of calcified cementum like
tissue, which is deposited directly on a tooth root.
Clinical presentation
Signs and symptoms:
 Hard expansion
 Deep, dull pain
Overlying mucosa is intact
VITAL TOOTH.
Associated teeth are not mobile
Site:
• In the premolar or molar region.
• Mandible > maxilla

Radiographically:
• Radiopaque mass fused to root and is surrounded by a thin radiolucent rim.

Differential diagnosis:
• The distinguishing features of a cementoblastoma are:
i. its peripheral radiolucent margin to adjacent bone
ii. and its obliteration of one half of the root.
Histopathology:
i. Calcified cementum like material with reversal
lines.
ii. The periphery of the lesion is uncalcified [that’s
why the lesion appears with a radiolucent rim in
radiographs].
iii. The fibrous stroma is highly vascular.

Treatment:
➢ Removal of the associated tooth with the lesion
attached.
➢ Incomplete removal of the lesion commonly
leads to recurrence.
C- Mixed Odontogenic Tumors

Benign:
1. Ameloblastic fibroma
2. Odontomas

Malignant:
Ameloblastic fibrosarcoma
Benign:
1-Ameloblastic fibroma
(ameloblastic fibrodentinoma, and ameloblastic fibro-odontoma)
• Is a benign tumor in which both the epithelial and mesenchymal elements are neoplastic (both are
proliferating).
• does not exhibit a locally invasive growth pattern.
Clinical Features:
➢ Age: more common in young patients (first two decades)
➢ Site: more common in the mandible (posterior premolar-molar area)
➢ Slowly growing, painless expansion of the jaws.
Radiographic Features:
✓ well-defined small unilocular radiolucency
✓ may multilocular with larger tumors
✓ may be associated with an impacted tooth
Histopathological Features:
▪ The tumor is usually surrounded by fibrous capsule.
▪ It consists of both active primitive mesenchyme and ameloblast like epithelial
components
• Note:
• Rarely, few lesions may show formation of hard dental tissues, these
are referred to as ameloblastic fibrodentinoma or ameloblastic fibro-
odontoma, but most likely they are developing odontomas.

Treatment and Prognosis:


➢ Enucleation and curettage.
➢ Low recurrence rate.
➢ Extensive destructive tumors should be treated radically
2. Odontomas
Odontomas are mixed epithelial and mesenchymal tumor-like
malformations (hamartomas) composed of dental hard and soft tissues.
➢ They are the most common types of odontogenic tumors
➢ Odontomas are further subdivided into compound and complex
types.
Clinical Features:
➢ Age: Children and adolescents
➢ Site: compound odontomas: more common in anterior
maxilla
complex odontomas: more common in molar region of
either jaws
➢ Appearance: The majority of these lesions are completely
asymptomatic, being discovered on routine radiographic
examination or when films are taken to determine the reason
for failure of a tooth to erupt.

Radiographic Features:
i. Compound odontoma will present a gravel-like
appearance in which the outline of miniature teeth may
be noticed surrounded by a narrow radiolucent zone.

ii. Complex odontoma will present as a dense amorphous


and irregularly shaped mass also surrounded by a
narrow radiolucent rim.
iii. An unerupted tooth is frequently associated with the
odontoma, and the odontoma prevents eruption of the
tooth.
Histopathological Features:
i. Compound odontoma: consists of multiple tooth-like structures (denticles) contained
in a loose fibrous matrix. Each denticle has an organized structure with pulp centrally
and an enamel cap over the abnormally shaped dentine.

ii. Complex odontoma: single irregular mass of dental tissues, having no morphological
resemblance to a tooth forming a disorganized structure of enamel and dentin.

Treatment:
➢ Enucleation and curettage.
➢ They are not adherent to bone and can be enucleated from the bony cavity with hand
curettes.
3-lnvaginated odontoma
(Dens invaginatus, Dens in Dente)
arise as a result of invagination of a portion of the enamel
organ into the dental papilla at an early stage in
odontogenesis, before the formation of calcified dental
tissues.
 The majority of invaginations originate in the coronal part
of the tooth but radicular invaginations also occur

Radiographically:
 A tooth-like structure appears within the pulp of the
involved tooth and hence the term “dens in dente” was used.

Clinically:
 The dens in dente may appear as either a normally shaped
or malformed crown that exhibits a deep pit or crevice in
the area of cingulum.
 Food debris tend to lodge in the pit causing caries, pulp
infection and periapical disease.
Degree of invagination :
 type 1, where the invagination is confined
to the crown of the involved tooth;
 type 2, where the invagination extends
into the root
 type 3, where the invagination extends
through the root apex.
4- Evaginated odontoma
Dens evaginatus
➢ appears as an accessory cusp on the occlusal surface between the
buccal and lingual cusps of premolars, unilaterally or bilaterally.
➢ It may rarely occur on molars, canines and incisors.
➢ A pulp horn may extend into this extra cusp.

Pathogenesis:
- Proliferation and evagination of enamel epithelium and subjacent
odontogenic mesenchyme during tooth development.

Clinical significance:
- The “extra” cusp may contribute to displacement of the teeth
resulting in malocclusion.
- Pulp exposure with subsequent infection following occlusal wear.
5- Enamel pearl
(enameloma)
• is a small, spherical enamel projection located on the
root surface near the bifurcation or trifurcation area
and is usually found on maxillary molars.

Histologically:
It may consist of enamel only or enamel, dentine and
pulp.

Pathogenesis:
1. Abnormal displacement of ameloblasts below
amelodentinal junction.
2. Differentiation of cells of the epithelial root sheath of
Hertwig into ameloblasts during root formation so that
a nodule of enamel forms in place of cementum.
ii- Malignant Mixed Odontogenic Tumors
Ameloblastic fibrosarcoma
➢ It is the malignant counterpart of ameloblastic fibroma. It is invasive
and destructive but has little tendency to metastasize.
➢ In these sarcomas the epithelial component remains benign, but the
mesenchymal component shows features of malignancy.
***Cemento-Osseous Dysplasias
1. Focal cemento-osseous dysplasia
2. Periapical cemento-osseous dysplasia
3. Florid cemento-osseous dysplasia
Cemento-Osseous Dysplasias

➢ They are non-neoplastic disturbances of growth


and remodeling of bone and cementum.

➢ They are the most common “fibro-osseous lesions”

➢ They have similar histological and radiological


features and differ mainly in their extent

➢ There are variable types of cemento-osseous


dysplasia’s including: focal cementoosseous dysplasia,
periapical cemento-osseous dysplasia and florid
cemento-osseous dysplasia

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