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ODONTOGENIC

TUMORS
OF THE JAWS

MADE BY: NAVNEET REHAL


MDS STUDENT
DEPARTMENT OF ORAL AND MAXILLOFACIAL SURGERY
ODONTOGENIC TUMORS OF THE JAWS

Odontogenic tumors comprise a group of lesions of the jaw derived from the
primordial tooth forming tissues (odontogenic epithelium) and presenting
a large number of histologic patterns.

These tumors, are found exclusively within the jawbones (intrabony or centrally
located) or in the soft mucosal tissue overlying tooth-bearing areas (peripherally
located). The tumors may be generated at any stage of an individual's life.

“Odontogenic tumors and tumor-like lesions constitute a group of heterogeneous


diseases that range from hamartomatous or non-neoplastic tissue proliferations to
benign malignant tumors with metastatic potential. They are derived from epithelial,
ectomesenchymal and/ or mesenchymal elements of the tooth forming apparatus.
Odontogenic tumors are rare, some even extremely rare, but can pose a significant
diagnostic and therapeutic challenge.” -( WHO 1992)

ODONTOGENESIS :
Odontogenic neoplasms, hamartomas, and odontogenic cysts are all lesions derived
from the tooth-producing apparatus, and as such they often reflect aspects of
odontogenesis.

The development of tooth occurs in the following stages:

1) Bud stage

2) Cap stage

3) Early bell stage

4) Late bell stage (formation of dental papilla )

Once the process of odontogenesis is completed, the dental lamina leaves behind
some cell remnants in the connective tissue, which are called the “cell rests of sarre”.

The remnants of the epithelial root sheath of Hertwig also remain within the
periodontal ligament and are known as “cell rests of Malassez”.

These cellular remnants play important role in the development of various


odontogenic tumors
CLASSIFICATION OF ODONTOGENIC TUMORS :

THOMA AND GOLDMAN 1946 :


 The lesions primarily derived from epithelium
 Those originated predominantly from mesenchyme
 A mixed group—both epithelial and mesenchymal tissue shared in the
formation of a lesion

CLASSIFICATION OF ODONTOGENIC TUMORS - GORLIN ( 1961)


EPITHELIAL ODONTOGENIC TUMORS :

A. Minimal inductive change in connective tissue (ectodermal origin)

 Ameloblastoma
 Adenomatoid odontogenic tumor
 Calcifying epithelial odontogenic tumor (CEOT)

B. Marked inductive change in connective tissue (mixed origin)

 Ameloblastic fibroma
 Ameloblastic odontoma
 Odontoma
 Complex odontoma
 Compound odontoma

MESODERMAL ODONTOGENIC TUMORS

 Odontogenic myxoma
 Odontogenic fibroma
 Cementoma

a. Periapical cemental dysplasia (PCD)

b. Benign cementoblastoma
c. Cementifying fibroma

d. Familial multiple (gigantiform) cementoma (Florid osseous dysplasia) (FOD)

KRAMER, PINDBORG, SHEAR CLASSIFICATION OF ODONTOGENIC TUMORS


(1992) :

A. Benign

I Odontogenic epithelium without odontogenic ectome senchyme

o Ameloblastoma
o Squamous odontogenic tumor
o Calcifying epithelial odontogenic tumor (Pind borg tumor)
o Adenomatoid odontogenic tumor*

II. Odontogenic epithelium with odontogenic ectom esenchyme with or without


hard tissue formation

o Ameloblastic fi broma
o Ameloblastic fi brodentinoma
o Ameloblastic fi bro-odontoma
o Odontoameloblastoma
o Calcifying odontogenic cyst
o Complex odontoma
o Compound odontoma

III. Odontogenic ectomesenchyme with or without in clu ded odontogenic


epithelium

o Odontogenic fi broma
o Myxoma (myxofi broma)
o Cementoblastoma (benign cementoblastoma, true cementoma)

B. Malignant

I. Odontogenic carcinomas

o Malignant ameloblastoma
o Primary intraosseous carcinoma
o Clear cell odontogenic carcinoma**
o Ghost cell odontogenic carcinoma

II. Odontogenic sarcomas


o Ameloblastic fi brosarcoma
o Ameloblastic fi brodentinosarcoma
o Ameloblastic fi bro-odontosarcoma

HISTOLOGICAL CLASSIFICATION OF ODONTOGENIC TUMORS

-WHO CLASSIFICATION ( 2005)

I. Benign

Odontogenic epithelium with mature, fibrous stroma; odontogenic


ectomesenchyme not present

Ameloblastoma

o Solid/multicystic
o Extraosseous/ perlpheral
o Desmoplastic
o Unicystic

Squamous odontogenic tumor

Calcifying epithelial odontogenic tumor

Adenomatoid odontogenic tumor

Keratinizing cystic odontogenic tumor

Odontogenic epithelium with odontogenic ectomesenchyme with or without


dental hard tissue formation

Ameloblastic fibroma

Ameloblastic fibrod entinoma

Ameloblastic fibro-odontoma

Complex odontoma

Compound odontoma

Odo ntoameloblastoma

Calcifying cystic odontogenic tumor

Dentinogenic ghost cell tumor

Mesenchyme and/ or odontogenic ectomesenchyme with or without included


odontogenic epithelium
Odontogenic flbrorna (epithelium-poor and epithelium-rich types)

Odontogenic myxoma or fibromyxoma

Cementoblastoma

Malignant tumors (odontogenic carcin omas)

Metastasizing, malignant ameloblastoma

Ameloblastic carcinoma

(a) primary

(b) seconda ry (dedifferentiated), intraosseous

(c) secondary (dedifferenti ated), extraosseous

Primary intraosse ous squamous cell carcinoma (PIOSCC)

(a) PIoscc solid type

(b) PIoscc derived from odontogenic cysts

(c) PIOSCC derived from kerat inizing

cystic odontogenic tumor

Clear cell odontogenic carcinoma

Ghost cell odontogenic carcinoma

Malignant tumors (odontogenic sarcomas)

Ameloblastic fibrosarcoma

Ameloblastic fibrodentino- and fibro-odontosarcoma

2017 WHO CLASSIFICATION OF ODONTOGENIC TUMORS :

Malignant Odontogenic Tumors

 Ameloblastic carcinoma
 Primary intraosseous carcinoma, NOS
 Sclerosing odontogenic carcinoma
 Clear cell odontogenic carcinoma
 Ghost cell odontogenic carcinoma
 Odontogenic carcinosarcoma
 Odontogenic sarcomas
Benign Odontogenic Tumors Epithelial Origin
 Ameloblastoma, conventional
 Ameloblastoma, unicystic type
 Ameloblastoma, extraosseous/ peripheral type
 Metastasizing (malignant) ameloblastoma
 Squamous odontogenic tumor
 Calcifying epithelial odontogenic tumor
 Adenomatoid odontogenic tumor

Mixed (Epithelial-Mesenchymal) Origin


 Ameloblastic fibroma
 Primordial odontogenic tumor
 Odontoma
 Compound type
 Complex type
 Dentinogenic ghost cell tumor

Mesenchymal Origin
 Odontogenic fibroma
 Odontogenic myxoma/myxofibroma
 Cementoblastoma
 Cemento-ossifying fibroma

GENERAL PRINCIPLES OF MANAGEMENT OF A JAW LESION


HISTORY OF THE LESION :

Duration:

Prolonged duration : may be congenital

Long duration without pain: benign neoplasm

Short duration, rapid growth : malignant growth

Mode of onset and progress:

History of trauma may be obtained in many bone lesions like osteogenic sarcoma.
Spontaneous swelling and rapid growing lesion may bemalignant, while very slowly
growing lesion may be benign growth.

Exact site and shape:

In a huge swelling it is essential to know the exact site and shape of the swelling for
knowing the origin of the lesion.

Progress of the lesion:


Whether the swelling has been growing slowly or it has remained stationary for a
long time (benign growth). Has it been growing again after a stationary period of
months/years (MT in a benign lesion) or has it been continuously increasing in size
(malignant growth)

Change in character of a lesion:

ulcerations over the lesions, Fluctuation, softening, etc. are noticed by the patient
recently, Whether painless swelling has become painful—secondary infection may
have set in the lesion.

Associated symptoms:

Pain, abnormal sensations, anesthesia, paresthesia over a region, dysphasia, nasal


obstruction—breathing difficulty, tenderness, lymphadenopathy. Restriction of the
oral opening or trismus—due to involvement of pterygoid plates by the lesion it can
be noticed.

CLINICAL EXAMINATION OF THE LESION

Thorough clinical examination of the lesion, as well as of the entire oral cavity and
external facial area including all regional lymph nodes is a must. The quick
assessment of general physical examination also should be noted down

ON INSPECTION :

_ Number—whether single or multiple lesions


_ Size
_ Site or anatomical location of the lesion— palatal
swellings may have salivary gland origin, and may not
have odontogenic origin
_ Shape and size of the lesion— whether ovoid, spherical, localized, diffuse, etc.
_ Color of the lesion—whether red or purple (hemangioma), blue (ranula)
_ Surface—whether smooth, lobulated (benign) or irregular, ulcerated, fungating
growth (malignancy)
_ Whether it is pedunculated or sessile?
_ Skin over the swelling—red, hot skin will suggest secondary infection.
ON PALPATION :
Palpation should be carried out gently and systematically.
Consistency of the lesion:
Soft (lipoma), hard or indurated, firm (fibroma), bony hard (osteoma), cystic, etc. It
should be also noted whether the consistency is uniform throughout or variable.
Whether there is egg shell crackling present? Fluctuation is present?
Presence of pulsations:
If the palpation of a lesion reveals a pulsatile quality, a large vascular component is
indicated. The “thrill” can be felt along the lesion. The auscultation with a
stethoscope may reveal “a bruit” or audible murmur.
Fixity:
Fixity to the overlying skin/mucosa/underlying structures.
Lymph node examination:
Systematic lymph node palpation should be carried out.

RADIOGRAPHIC DIAGNOSIS OF ODONTOGENIC TUMORS :


• INTRA-ORAL RADIOGRAPHS
• EXTRA-ORAL SPECIAL PROJECTIONS
• DENTAL PANORAMIC RADIOGRAPHS
• COMPUTED TOMOGRAPHY
• MAGNETIC RESONANCE IMAGING
• RADIONUCLIDE IMAGING

HISTOPATHOLOGIC EXAMINATION OF THE LESION :


Exfoliative cytology:
in case of suspected malignancy of the jaw bone, the initial screening can be done
by scraping the lesion.
Aspiration biopsy:
It is done by using a needle and syringe to penetrate the lesion and aspirating the
contents of the lesion.
Aspiration can be carried out for all the lesions, which contain fluid or for any
intraosseous lesion prior to surgical intervention.
Aspiration can be done by using 18-G needle with 5/10 cc syringe. material obtained
from the aspiration biopsy is submitted for
(1) pathological examination
(2) chemical analysis or
(3) microbiologic culturing if necessary.

Fine-needle aspiration cytology (FNAC):


It can be useful for deep seated lesions, With Silverman needle, it is possible to get
a strip of intact tissue 1.5 mm wide and about 1.5 cm long, which can be sectioned
and studied like an ordinary surgical biopsy.

Excisional biopsy:
This is the complete removal of the pathological lesion, which is submitted for
histopathological review. Some amount of normal surrounding tissue is also included
during total excision of the lesion. It is usually employed for smaller lesions, which
can be excised easily without causing much mutilation.

Incisional biopsy:
In the larger lesions, the small wedge of tissue is obtained to represent the entire
lesion and subjected for histopathological examination.

Tumors Arising from Odontogenic Epithelium without Odontogenic


Ectomesenchyme :
AMELOBLASTOMA :
(Adamantinoma, adamantoblastoma, multilocular cyst)
The ameloblastoma is a true neoplasm of enamel organ type tissue which does not
undergo differentiation to the point of enamel formation.
Robinson described ameloblastoma as :
"as being a tumor that is ‘usually unicentric, nonfunctional, intermittent in
growth, anatomically benign and clinically persistent’.
It was first recognized by Cusack in 1827
it was named ‘adamentinoma’ in 1885 by LuisCharles Malassez.
It was finally renamed as ‘ameloblastoma’ in 1934 by Ivey and Churchill

ETIOLOGY
the following factors may predispose the formation of ameloblastoma:
• Trauma
• Infection
• Previous inflammation
• Extraction of tooth
• Dietary factors
• Viral infection.
PATHOGENESIS :
According to Thoma, Williams in 1993, the tumor may be derived from various
origins.

 Late developmental sources: Cell rests of enamel organ, either remnants of


dental lamina or epithelial cell rests of Malassez or remnants of Hertwig’s
sheath, follicular sacs.
 Early embryonic sources: Disturbances of developing enamel organ, dental
lamina, tooth buds.
 Basal cells of the surface epithelium of the oral mucosa.
 Secondary developmental sources: Epithelium of odontogenic cysts,
particularly primordial, lateral periodontal cyst, dentigerous cyst and
odontomas.
 Heterotropic epithelium in other parts of body, especially from the pituitary
gland.
CLINICAL FEATURES
Incidence: Approximately one percent among all oral tumors and 18 % of all
odontogenic tumors are ameloblastomas.
Age: Second, third, fourth and fifth decade of life, the mean age of occurrence is
about 32 years. This lesion occurs more commonly in blacks than whites.
Sex: Males are affected more often than females.
Site: Ameloblastoma in most of the cases involve the mandible (80%), especially in
the molar-ramus area (70%), although some lesions may develop in the premolar
(20%) or symphysis (10%) regions.
Maxillary tumors also commonly involve its posterior part and the lesions often have
a tendency to invade into the antrum (15%) or the nasal floor.
Extraosseous or peripheral ameloblastomas can rarely occur mostly in relation to the
gingiva.
RADIOGRAPHIC CONSIDERATIONS :
Radiographically ameloblastoma usually presents as

 a well-defined, multilocular, radiolucent area in the bone with a typical “honey-comb” or


“soap-bubble”appearance Few lesions can be unilocular too.
• For multilocular lesions, when the loculations are large the appearance will be ‘soap bubble’
type and when the loculations are small the appearance will be ‘honey-comb’ type.
• The larger lesions often cause expansion, distortion or even perforation of buccal and lingual
cortical plates
• In radiograph, the lesion typically exhibits an irregular and “scalloped” margin
• Resorption of roots of the adjoining normal teeth is often seen in rapidly growing lesions.
• Ameloblastoma can cause expansion of the lower border of mandible.
• As the neoplasm progresses, it sometimes become associated with an impacted tooth
(mostly the third molars) and in such cases the lesion may resemble a dentigerous cyst.
• Desmoplastic ameloblastoma presents a mixed radiolucent and radiopaque appearance,
thus often resemble a fibrosseous lesion.

PATHOLOGIC FEATURES :

Histologically ameloblastoma shows neoplastic proliferation of odontogenic epithelial


cells (ameloblast-like cells), the cells exhibit their nucleus moved away from the
basement membrane and this particular phenomenon is called ‘reverse
polarization’.

Histologically ameloblastomas present two distinct patterns:

(i) Plexiform

(ii) Follicular pattern,


(iii) other relatively uncommon histological patterns namely the acanthomatous
pattern, basal cell pattern, granular cell pattern and desmoplastic pattern

Follicular type (resembles tooth follicle): It consists of small to large odontogenic


epithelial nests (the follicles) and variously shaped and sized ameloblastomatous
islands (more common pattern). Cyst formation is commonly seen
• Subtypes of Follicular Ameloblastoma-
• Acanthomatous type : compression of stellate reticulum into a squamoid
mass with squamous metaplasia is seen.
• Basal cell type : bears resemblance to the basal cell carcinoma of the skin.
The inner follicular and outer follicular cells may assume a basal cell
appearance throughout the lesion
• Granular cell type— stellate reticulum like cells, get transformed into cells
with coarse granular, eosinophilic cytoplasm. This type is found to be more
aggressive type with high chances of recurrence and metastasis (MT)

The plexiform type: It consists of interlacing strands of narrow or wide odontogenic


epithelial trabeculae resembling the dental lamina.
OTHER SUBTYPES :
• Desmoplastic type :variant of connective tissue– stroma seen in solid
ameloblastoma. Small islands and cords of odontogenic epithelium in a
densely collagenized stroma.
• The mural ameloblastoma— another name for unicystic lesion, which is
found in children and young adults. The ameloblastic epithelium lines the
laminal surface and may proliferate into the cyst (mural growth)
• The peripheral ameloblastoma—origin is from residual or displaced
odontogenic tissue in the gingiva. This lesion is not encapsulated like the
intrabony ameloblastoma, but is circumscribed by stromal connective tissue
through which it can infiltrate into the surrounding mucosa as well as
penetrate the periosteum and invade the bone
• Malignant subtypes—the term “malignant ameloblastoma” is applied, when
the metastatic tumor resembles primary tumor with no histological
transformation.

MANAGEMENT :
The basic modality of the management of the ameloblastoma is surgery. Wide local
excisions with at least 1 to 1.5 cm margins of normal bone are recommended
recommended for the central tumors. The tumor is known to recur after incomplete
excision.- Fonseca
ENUCLEATION AND CURRETAGE :
Curettage should never be considered as the treatment modality, since intraosseous
multicystic lesions ‘recurrence rate is 55–100% after curettage, and for intraosseous
unicystic lesions— 18–25%.

RESECTION OF THE MANDIBLE :


Resection with continuity: Marginal mandibulectomy or wedge
mandibulectomy is the method of maintaining the continuity of the bone at the same
time excising the lesion.
The ameloblastoma tends to infilterate the cancellous bone and the thick cortical
plates are eroded but not invaded.
The mandibular anterior has plenty of cortical boneand the ameloblastomas, which
are relatively small and have not eroded the cortical bone significantly can be treated
with marginal mandibulectomy.
The large lesions whichhave severely undermined the cortex and are likely to sustain
a pathological fracture require segmental resection.

Resection without continuity: Segmental resection —when the tumor


has grown sizably and the inferior border of the mandible is severely undermined the
segmental resection is recommended
The attempts to preserve the inferior border may compromise the surgical clearance
and the chances of recurrence and pathologic fracture are very high.

Hemimandibulectomy: A classical hemimandibulectomy comprises of resection


of half of the mandible along with the disarticulation of the condyle.
When an ameloblastoma involves the ramus extensively and surgical clearance is
doubtful thenthe classical hemimandibulectomy with disarticlation of the conyle is
undertaken
Preserving the condyle by resecting the tumor at subsigmoid level helps in better
reconstruction as the implant can be secured to the resected proximal fragment.

DIFFERENTIAL DIAGNOSIS :
Odontogenic keratocyst (primordial cyst)
• Dentigerous cyst
• Central giant cell granuloma
• Central hemangioma
• Aneurysmal bone cyst
• Pindborg’s tumor
• Fibromyxoma.

SURGICAL MANAGEMENT OF THE MAXILLARY TUMORS :

Jackson and Callon Forte (1996) have given guide lines depending upon
anatomical extents:
■ Tumor confined to maxilla without orbital floorinvolvement— partial maxillectomy
■ Tumors involving the orbital floor, but not the periorbital area—total maxillectomy.
■ Tumor involving orbital contents—total maxillectomy with orbital exenteration
■ Tumor involving the skull bone—along with skull base resection—neurosurgical
procedure.

RECONSTRUCTION OF THE SURGICAL DEFECT :


The reconstruction should ideally be done primarily to prevent deformity and collapse
of the resected segments due to fibrosis.
Reconstruction of the surgical defect is mandatory to prevent postoperative
morbidity.
As the majority of the patients are young, the reconstruction should fulfill following
goals:
• Restoration of movements and equilibrium of mandible
• Maintenance of normal occlusal plane, floor of the mouth and tongue
anatomical position
• Restoration of near normal feeding
• Acceptable esthetics and function
• More favorable social acceptance
The various modalities for reconstructing the surgical defect in the mandible are:
Reconstruction plates:
The metallic reconstruction plates made up of titanium or stainless steel are
commonly used.
The plates effectively bridge the bony defect. The plates are contoured to match the
facial profile and secured to the distal and proximal bony fragments with the help of
the screws
Bone grafts:
Corticocancellous bone grafts harvested from the anterior iliac crest or the rib grafts
can also be used to reconstruct the mandible
Free microvascular fibular grafts:
Anastamosed to regional artery and vein, they are the best option to reconstruct the
mandible and in selected cases maxillary defects
Custom made implants:
Using the stereolithography and rapid prototyping the custom made implants can be
fabricated and used to reconstruct the surgical bony defects.
It provides better results as compared to the reconstruction plate.
Bone transport:
The transport technique using the Elizarow technique by means of distraction
devices can be used to bridge smaller bony defects

RESCONSTRUCTION OF THE MAXILLARY DEFECTS :


The common modalities for maxillary reconstruction after the maxillectomy are:
• Use of temporalis muscle fascia flaps
• Use of nasal septum to reconstruct the orbital floor.
OBTURATORS :
The ensuing surgical defect following the maxillectomy performed for excising the
ameloblastoma can be plugged using prosthesis in the form of an obturator.

CALCIFYING EPITHELIAL ODONTOGENIC TUMOR (CEOT)


The calcifying epithelial odontogenic tumor is a locally aggressive neoplasm, which
is also known as Pindborg’s tumor (named after Prof JJ Pindborg, who first reported
it).
ORIGIN :
The lesion arises from either the cells of the stratum intermedium of the enamel
organ or the reduced enamel epithelium or even the remnants of the denral lamina.
Its biologic behavior is similar to that of ameoblastoma. However, it differs from
ameloblastoma by the fact that it is composed of spherical cells and not the
ameloblast-like cells as seen in ameloblastoma.
Site: Mandible is most commonly involved in the molar region (two-thirds of the
tumors). One-third of the tumors are found in the maxilla.
Relative distribution of CEOT, in the jaws is as follows– Maxilla– 8 % in anterior and
premolars, 21% in posterior region. Mandible– 14% in anterior and premolar, 57% in
molar region.
Fifty percent of the tumors are associated with an unerupted or embedded tooth
CLINICAL FEATURES :
The tumor usually presents a slow enlarging, painless swelling of the jaw with
expansion and distortion of the cortical plates.
• The swelling is usually bony hard and clinically it can be either well defined or
diffuse in nature.
• Displacement of regional teeth, with derangement of occlusion and facial
asymmetry, etc. are commonly present.
• Pain, paresthesia and other related symptoms may develop on rare occasions,
and few lesions may be even completely asymptomatic.
• Large maxillary lesions may invade into the antrum or the nasal floor and such
lesions occasionally cause nasal airway obstruction, epistaxis and proptosis of the
eyeball, etc.
• Extraosseous or peripheral lesions may cause nonspecific, sessile, superficial soft
tissue swellings of gingiva either in the tooth bearing or the edentulous areas of the
anterior jaw

RADIOLOGICAL FEATURES :
Radiographically calcifying epithelial odontogenic tumor usually presents a
welldefined, multilocular (rarely unilocular) radiolucent area in the jaw.
• Calcifications within the tumor is a characteristic finding in the calcifying epithelial
odontogenic tumors and radiographically it often exhibits multiple, small, radiopaque
foci of varying radiodensity within the radiolucent zone produced by the tumor
• This type X-ray of calcification within the tumor often produces a typical “driven
snow” appearance.
PATHOLOGIC FEATURES :

(1) Intraosseous-central—maxillary/mandibular posterior region


(ii) Extraosseous-peripheral— nonspecific, sessile gingival masses commonly seen
in anterior gingiva.
DIFFERENTIAL DIAGNOSIS
• Calcifying epithelial odontogenic cyst
• Adenomatoid odontogenic tumor
• Poorly differentiated carcinoma
• Ameloblastoma
• Ameloblastic fibro-odontome
• Dentigerous cyst
• Central ossifying or cementifying fibroma
SURGICAL MANAGEMENT :
Small, intrabony mandibular lesions with well-defined borders may possibly be cured
by simple complete, but conservative tumor enucleation or curettage followed by
judicious removal of a thin layer of bone adjacent to the tumor.
SQUAMOUS ODONTOGENIC TUMOR :
Squamous odontogenic tumors are rare, sometimes multifocal, potentially
aggressive neoplasms derived from the odontogenic epithelium
ORIGIN :
This neoplasm was first reported in the year 1975 and it probably originates from the
following tissue remnants found on the lateral root surfaces of the erupted teeth:
• Remnants of dental lamina.
• Cell rests of Malassez.
CLINICAL FEATURES
Age: It occurs more commonly among young adults.
Sex: Female predilection.
Site: Maxillary incisor-canine area and mandibular molar area. Sometimes the
neoplasm can be multicentric.
SIGNS AND SYMPTOMS :
Initially, there can be a painless swelling on the gingival areas of the jaw with mobility
and looseness of the regional teeth
• Intraosseous lesions are usually small and slow enlarging.
• There can be local tenderness in the area upon palpation.
• Many lesions are asymptomatic and are discovered incidentally during
radiographic examinations.
RADIOGRAPHIC FEATURES :
• Radiographically, squamous odontogenic tumor presents a well-circumscribed,
often semilunar or triangular shaped, unilocular radiolucent area with sclerotic
border.
• These lesions are interspersed between contagious teeth near their roots.
• Some lesions can be multilocular.
• Resorptions of root are usually absent.
• Some lesions of squamous odontogenic tumors mimic chronic periodontitis due to
similar type of extensive bone loss.
HISTOPATHOLOGY : Microscopically, squamous odontogenic tumor presents
irregularly-shaped, islands of welldifferentiated squamous epithelium in a mature
fibrous connective tissue stroma
DIFFERENTIAL DIAGNOSIS
• Acanthomatous ameloblastoma
• Lateral periodontal cyst
• Squamous cell carcinoma
• Central ossifying or cementifying fibroma
• Histiocytosis-X
• Collateral type of keratocyst.
SURGICAL MANAGEMENT :

Enucleation, curettage and local excision are treatment modalities that most
often are described in case reports of squamous odontogenic tumor. Clinically,
aggressive lesions have been treated by en bloc excision.

CLEAR CELL ODONTOGENIC TUMOR :


Clear cell odontogenic tumor is a locally aggressive, rare odontogenic neoplasm of
epithelial cell origin.
CLINICAL FEATURES

Age: Above 50 years of age.

Sex: More prevalent among females.

Site: Both maxilla and mandible can be affected.


CLINICAL PRESENTATION Clinical features are not pathognomonic and both jaws can be
affected by the tumor.

Clinically there can be mild pain and swelling of the jawbone with expansion of the cortical
plates and mobility of the teeth.

RADIOLOGICAL FINDING The tumor produces irregular radiolucent area in the bone with
a ragged border.

HISTOLOGICAL PRESENTATION Histologically this neoplasm is poorly circumscribed and


it consists of sheets of odontogenic epithelial cells with clear cytoplasms and centrally placed
nuclei.

SURGICAL TREATMENT : Surgical excision and curettage.

ADENOMATOID ODONTOGENIC TUMOR :


The adenomatoid odontogenic tumor is a relatively uncommon, well-circumscribed,
odontogenic neoplasm characterized by the formation of multiple ‘ducts-like’
structures by the neoplastic epithelial cells.
The name ‘adenomatoid’ has been given to the neoplasm because histologically
numerous ductlike structures are often interspersed throughout the lesion, which
gives a glandular or adenomatoid appearance to it
ORIGIN :
The tumor probably arises from the reduced enamel epithelium, during the
presecretory phase of enamel organ development.
it may develop from either the dental lamina or from a pre-existing dentigerous cyst.
CLINICAL FEATURES :
Age: The tumor usually occurs in the younger age (e.g. second and third decade of
life).
Sex: Females are more commonly affected in comparison to the males, with a ratio
of 2:1.
Site: The lesion most typically occurs in the maxillary anterior region (upper lateral
incisorcanine area). Sometimes it can occur in the premolar region. Rarely does it
involve the mandible in the angle-ramus area.
In about 70% cases, the neoplasms occur in association with an unerupted tooth.
Some lesions develop extraorally in relation to the gingiva
The tumor usually presents a slow enlarging, small, bony hard swelling in the
maxillary anterior region
RADIOGRAPHIC FEATURES :
Radiographically, adenomatoid odontogenic tumor presents a well-circumscribed,
unilocular, radiolucent area, which often encloses a tooth or tooth-like structure
(mostly maxillary canine)
• Multiple small, radiopaque foci of varying radiodensity may be present inside the
lesion and the finding is known as ‘snow-flake’ calcifications.
• The border of the lesion is not well corticated and it consistently engulfs the
impacted tooth including its root. This feature differentiates adenomatoid
odontogenic tumor from dentigerous cyst, since the later lesion encloses only the
crown portion of an impacted tooth.
PATHOLOGIC FEATURES :
Microscopically, adenomatoid odontogenic tumor reveals spindle shaped, neoplastic
odontogenic epithelial cells proliferating in multiple “duct-like” patterns, within a thin
but well-vascularized stroma.
Foci of calcification presumed to be abortive enamel formation or /cementum like
material are seen.
Calcification in several forms may be observed.
■ Irregular dystrophic bodies
■ Laminated or ring like calcifications
■ Large globular masses.
SURGICAL MANAGEMENT :
Enucleation and Curettage: As the tumor is well encapsulated and usually present in
the anterior maxilla, the surgical access is simple. The tumor is easily shelled out
from the bone and in most of the cases can be enucleated in total.
TUMORS ARISING FROM ODONTOGENIC EPITHELIUM WITH ODONTOGENIC
ECTOMESENCHYME WITH OR WITHOUT DENTAL HARD TISSUE FORMATION
AMELOBLASTIC FIBROMA :
Ameloblastic fibroma is a true benign odontogenic tumor in which both the epithelial
and the mesenchymal elements are neoplastic (In ameloblastoma only the
epithelium is neoplastic).
Ameloblastic fibroma and related lesions were defined by the WHO as:
Ameloblastic fibroma consists of odontogenic ecto-mesenchyme resembling the
dental papilla and epithelial strands and nests resembling dental lamina and enamel
organ.
• No dental hard tissue is present.
• If there is dentin formation, the lesion is referred to as ameloblastic fibrodentinoma.
CLINICAL FEATURES :
Age: It occurs usually below the age of 20 years (average age 14 years).
Sex: More often in males than females.
Site: Mandibular posterior (premolar-molar) region is the most common site,
maxillary tumors are usually rare.
CLINICAL PRESENTATION
• A slow growing, painless, bony hard swelling of the jaw.
• Few lesions can be completely asymptomatic and discovered accidentally during
routine examinations.
• There may be mobility of the regional teeth, with obvious facial asymmetry.
• The tumor develops predominantly above the impacted or unerupted molar teeth
as a pericoronal lesion.
• Over 90 percent cases it is associated with an impacted tooth.
• Untreated lesions can be exceptionally large.
RADIOGRAPHIC FEATURES :
On radiograph, the tumor usually presents a well-defined, unilocular or multilocular
radiolucent area that often resembles ameloblastoma or dentigerous cyst.
Lesions are well corticated and they vary considerably in size.
HISTOPATHOLOGICAL FEATURES :
Ameloblastic fibroma arises from odontogenic epithelial and mesenchymal cells of
the enamel organ and dental papilla, respectively.
It appears to be a true mixed tumor and it is noticeable that, unlike the histologically
somewhat similar, appearing ameloblastoma, malignant change in ameloblastic
fibroma affects the mesenchymal component and not the epithelium.
SURGICAL TREATMENT :
By surgical excision with thorough curettage of the bone. Recurrence is uncommon.

AMELOBLASTIC FIBRO-ODONTOMA :
It is a benign neoplasm of odontogenic origin and is characterized by the presence of
combined features of ameloblastic fibroma along with the presence of calcified
enamel or dentin like tissues
The revised WHO classification of odontogenic tumor defines ameloblastic fibro-
odontoma as “a lesion similar to ameloblastic fibroma, but showing inductive
changes that lead to formation of dentin and enamel.”
CLINICAL FEATURES :
Age: Children and young adults.
Sex: No predilection.
Site: Posterior region of jaw, more common in mandible.
CLINICAL PRESENTATION :
• Most of the lesions are asymptomatic.
• Lesions are often associated with an impacted or missing tooth
• Larger lesions cause progressive, painless swelling of the jaw with expansion
of the cortical plates.
• Untreated lesions can result in severe facial deformity.
RADIOLOGICAL APPEARANCE :
• Radiographs show unilocular radiolucent area (rarely multilocular) containing
multiple radiopaque foci having radiodensity similar to that of tooth.
• a solid conglomerated mass is seen inside the lesion, unerupted tooth is often
present near the lesion or sometimes the crown of the tooth is included in the
lesion.
• Early lesions may be comparatively radiolucent as the calcified tissues were
yet to form.
Histopathologic features
The tumor is composed of strands, cords and islands of odontogenic epithelium
distributed in a cell-rich, dental papilla like ectomesenchymal stroma. Varying
amounts of osteodentin or dentin-like material and occasionally enamel matrix can
be identified.

SURGICAL TREATMENT :
Conservative surgical enucleation is considered to be the treatment of choice
with concurrent removal of the associated unerupted tooth. Extensive lesions
causing severe undermining of the bone require segmental resection of the mandible

AMELOBLASTIC FIBRODENTINOMA :

Ameloblastic fibrodentinoma is defined‘a neoplasm similar to ameloblastic fibroma,


but also showing inductive changes that lead to the formation of dentin.’ It is thought
that ameloblastic fibrodentinoma is a histologic variant of ameloblastic fibroma in
which dentin or dentinoid tissue has formed.
Considered to be a hamartoma rather than a neoplasm

CLINICAL FEATURES
Site : more common in mandible than maxilla (3:1)
Sex : in males more often than females (2:1)
Age : 4–60 years of age (mostly below 30 years of age)
• In children the lesion may be associated with unerupted or missing deciduous
teeth and found inthe anterior jaw.

• In adult : seen more in posterior region involving permanent teeth


• Asymptomatic swelling of the jaws

RADIOGRAPHIC FEATURES :
small or extensive uni- or multilocular with well-defined borders. Radiopacity may be
dispersed throughout the lesion. The associated teeth are often seen to be deeply
situated in the lesion
PATHOLOGIC FEATURES :
Histologically— several configurations are seen within a basic lesional format of
ameloblastic fibroma.
Various stages of induction of dentin may be demonstrated, resulting in lesions that
display dentinoid, osteodentin and rarely, tubular dentine. The dentine may be
infrequently and poorly mineralized

SURGICAL TREATMENT: complete excision of the lesion.

ODONTOMA :
Odontomas are a group of common hamartomatous odontogenic lesions with limited
growth potential. These lesions are capable of producing normal appearing enamel,
dentin, cementum and pulp, etc. in an unorganized fashion

ORIGIN :
The tumor probably occurs during the development of teeth at a point after the stage
of histodifferentiation but prior to the stage of morphodifferentiation.

TYPES OF ODONTOMAS
Two types of odontomes are commonly recognized:
A. Compound odontome, and
B. Complex odontome (both these lesions are closely related malformation).

Complex odontoma : It consists of a completely disorganized and diffuse mass of


odontogenic tissue with haphazardly arranged enamel, dentin and cememtum.

Compound odontoma : Compound odontome presents collections of numerous


small, discrete, tooth-like structures. Most odontogenic tissues in compound
odontome bear superficial anatomical resemblance to normal teeth.

CLINICAL FEATURES :

Frequency: Odontomes represent about 7% of all odontogenic neoplasms.


Age: These lesions usually occur among children or young adults (preferably in their
second decade of life).
Sex: Both sexes are almost equally affected or there may be a slight male
predominance.
Site: Maxilla is more commonly affected than the mandible. Odontomes are most
commonly seen in the pericoronal area of the permanent teeth.
• Compound odontomes usually involve the anterior part of the maxilla
(intercanine area).
• Complex odontomes often involve the posterior (premolar-molar) region of the
jaw and are slightly more common in mandible. Some lesions may occur
extraosseously in the gingival soft tissues.
CLINICAL PRESENTATION
• Odontomes generally produce small, asymptomatic lesions, which are detected
incidentally.
• The lesions vary in size greatly, it can be as small as a tooth or it may be as large
as six centimeters in diameter.
• In few cases, they may produce large, bony hard swellings of the jaw, with
expansion of the cortical plates and displacement of the regional teeth
A tooth may be often missing from the dental arch as the odontome can block the
eruption path of the tooth.
• The lesions are often associated either with an impacted tooth or a retained
deciduous tooth.
• If the odontomes are located high in the alveolus, they may tend to erupt in the oral
cavity by resorbing the overlying bone and as a result there may be pain,
inflammation, ulceration or fistula formation, etc.
• Multiple odontomes can occur in the jaw simultaneously in some patients.
• Some odontomes may exhibit cyst formation around the tumor mass (mostly
dentigerous cyst).

RADIOGRAPHIC FEATURES :
Odontomes usually produce pericoronal radioluncencies with well-defined and well-
corticated borders.
A developing odontome may look completely radiolucent as the calcified elements
do not form in the initial stages.
The compound odontome:
• The compound odontomes radiographically appear as numerous, small,
miniature teeth or tooth-like structures, which are projecting from a single
focus
• they look like “a bag of teeth” and are commonly located between the roots
of the erupted permanent teeth or above the crown of an impacted tooth.
The complex odontome:
• The complex odontomes radiographically appears as round or oval or
‘sunburst-like’, conglomerated radiopaque mass within the jawbone.
• They do not produce any morphologically identifiable tooth or tooth-like
structures.
• Both types of odontomes are usually surrounded by a thin radiolucent zone at
their periphery, which represents the capsule.
• When odontomes are associated with any impacted tooth, they are usually
mandibular molars.
• Small odontomes may be located between the roots of the erupted teeth.
DIFFERENTIAL DIAGNOSIS
• Calcifying epithelial odontogenic tumor (CEOT)
• Ameloblastic fibrodentinoma
• Ameloblastic fibro-odontome
• Osteoma
• Odontoameloblastoma
• Focal sclerosing osteomyelitis.
SURGICAL TREATMENT :
Completely calcified complex odontoma is biologically inert and can be left alone
But, in case of pressure or pain symptoms or facial asymmetry, excision can be
planned. Care is taken to avoid pathological fracture during excision.

TUMORS ARISING FROM ODONTOGENIC ECTOMESENCHYME


WITH OR WITHOUT INCLUSION OF ODONTOGENIC EPITHELIUM

ODONTOGENIC FIBROMA :
Odontogenic fibroma is a rare benign neoplasm derived from connective tissue of
odontogenic origin and it can be either a peripheral lesion or a central (intraosseous)
lesion.

PERIPHERAL ODONTOGENIC FIBROMA :


Peripheral odontogenic fibroma is the most common form of the disease, which
develops extraosseously from the tooth bearing areas of the jaw
ORIGIN :
The lesion probably arises from the overlying gingival epithelium or the cell rests of
the dental lamina
CLINICAL FEATURES :
• Peripheral odontogenic fibroma clinically appears as a slow enlarging, exophytic,
well circumscribed, sessile growth of the gingiva
• The lesion is usually firm in consistency, it is painless and the overlying epithelium
is of normal color.
• Most of the lesions occur on the facial gingiva and their size ranges between 0.5 to
1.5 centimeter in diameter.
• Sometimes, more than one lesion can occur in the oral cavity but rarely.
• In some cases, there can be erythematous changes or even ulcerations on the
surface epithelium, which result from trauma.
• Interdental lesions may cause separation of the teeth.
• Consistency of the lesion often varies, since lesions may occur with ossification or
without ossification

RADIOGRAPHIC FEATURES :
• Since peripheral odontogenic fibromas are small lesions and they occur
extraosseously within the gingiva, radiographic changes in the bone are not
apparently found.
• However in certain cases saucerization of the cortical bone or widening of the
periodontal ligament space at the cervical region may be seen.
• Numerous foci of small radiopaque masses are sometimes found within few
lesions of peripheral odontogenic fibroma, which indicate calcifications within the
tumor

DIFFERENTIAL DIAGNOSIS
• Peripheral ossifying fibroma
• Peripheral giant cell granuloma
• True fibroma
• Neurofibroma
• Fibroepithelial polyp

SURGICAL TREATMENT : excision with a margin of uninvolved


tissue.
CENTRAL ODONTOGENIC FIBROMA
Central odontogenic fibroma is a relatively uncommon odontogenic neoplasm arising
within the jawbone.

CLINICAL FEATURES
Age: Wide age range (mean age is 20 years).
Sex: Female predilection is seen. Site: It occurs more often in relation to mandible
than maxilla. Maxillary lesions mostly occur anterior to the first molar tooth
Mandibular lesions generally occur in the posterior part of the jaw, in the tooth
bearing areas.

SIGNS AND SYMPTOM S :


• Central odontogenic fibroma produces a slow enlarging, non-descript, painless
swelling of the jaw.
• Displacement of teeth or formation of space or gap between teeth is common.
• Cortical expansion is often minimum, however larger lesions can cause localized
bony expansion and loosening of teeth .
• A large number of lesions can occur in association with unerupted teeth and
moreover some smaller lesions often remain asymptomatic.

RADIOGRAPHIC FEATURES
• Radiographically, the lesion presents a wellcircumscribed, rounded, unilocular
radiolucent area in the jaw.
• Some lesions are multilocular with sclerotic borders.
• Lesions often contain several small radiopaque flecks of varying radiodensity.
• Resorption of roots of the adjoining teeth is often seen in this tumor.

HISTOLOGICAL PRESENTATION : Histologically central odontogenic fibroma


presents a cellular connective tissue, containing numerous thin strands of
odontogenic epithelium

DIFFERENTIAL DIAGNOSIS
• Calcifying epithelial odontogenic tumor (CEOT)
• Ameloblastoma
• Cementifying fibroma
• Calcifying epithelial odontogenic cyst
• Central giant cell granuloma.
TREATMENT Surgical excision and curettage.

ODONTOGENIC MYXOMA
Odontogenic myxomas are aggressive, intraosseous neoplasms derived from
embryonic odontogenic mesenchyme.

ORIGIN :Odontogenic myxomas probably arise from the dental papilla or follicular
mesenchyme.

CLINICAL FEATURES
Age: Young and middle aged adults.
Sex: Both sexes are equally affected.
Site: Nearly all lesions are found in the tooth bearing areas of maxillary and
mandibular bone.
• Mandibular lesions are commonly found in the premolar-molar area. Some lesions
may be found in the ramus of the mandibular bone or other non tooth-bearing areas.
CLINICAL FEATURES :
• Odontogenic myxomas are slow growing but locally aggressive lesions, which often
cause painless swellings in the jaw
• Sometimes they cause displacement of the regional teeth.
• If left untreated, their size can be huge and that may cause considerable
expansion of the jaws with facial asymmetry.
• Maxillary lesions can perforate the bone and spread into the sinus. Afterwards they
can cross the midline septa and invade into the opposing sinus cavity.
• Mandibular lesions also often extend into the ramus area.
• Few lesions are asymptomatic and are detected only during routine radiographic
examinations.

RADIOGRAPHIC FEATURES
• The lesions often produce multilocular radioluency with a “soap bubble” or “honey
comb” appearance in the bone.
• Thin and extremely delicate septa of residual bone are often seen to course
through the radiolucent area
• These wispy trabeculae of thin bones are often arranged at right angles to one
another and thus they produce a “spider-web” like or “tennis racket” like appearance.
• Displacement of teeth is common and often there is root resorption in the adjacent
teeth.
• Border of the lesion is mostly ill-defined, irregular and scalloped.
• Smaller lesions of myxoma often appear as unilocular nonspecific radiolucent areas
in the jawbone.

DIFFERENTIAL DIAGNOSIS
• Central giant cell granuloma.
• Aneurysmal bone cyst.
• Chondromyxoid fibroma
• Ameloblastoma
• Calcifying epithelial odontogenic tumor
• Hyperparathyroidism
• Central neurilemmoma.

SURGICAL TREATMENT :
Since myxomas consist of a grossly gelatinous or mucoid material, which often
penetrate the surrounding trabacular spaces of bone, surgical excision of the lesion
and curettage is not always successful and hence resection of the jaw should be
done in larger lesions

CEMENTOBLASTOMA
Cementoblastoma is a rare benign odontogenic neoplasm arising from the
cementoblast cells. The tumor develops as an irregular rounded mass in continuity
with the apical cemental layer of a vital molar or premolar tooth.

CLINICAL FEATURES
Age: Usually second and third decade of life (Peak age of incidence is about 19
years).
Sex: Seen more frequently among males.
Site: Mandible is affected more often (75 percent cases) than the maxilla and
posterior part of the jaw is usually the site of choice for this tumor.

ORIGIN Cementoblastoma or “true cementoma” is a true neoplasm of the cemental


tissue. It arises from the cementoblast cells of the cemental layer of the apical third
of a vital tooth.
CLINICAL PRESENTATION
• Cementoblastoma often produces a slow enlarging, bony hard swelling of the jaw
that only rarely causes expansion of the jaw and displacement of the regional teeth
• Both buccal and lingual cortical plates are expanded uniformly.
• In most of the cases, low-grade intermittent pain may be present, which is felt more
often, when the area is palpated.
• The lesion is often attached to the apical third of a vital premolar or molar tooth.
Mandibular first molar is predominantly involved (50 percent cases).
• A dull sound is produced when the tooth percussed.

RADIOLOGICAL FEATURES
• Radiographically the tumor often presents a large, dense, radiopaque mass that is
often attached to one or more vital tooth roots. The early lesions may be radiolucent.
• Resorption and subsequent fusion of the tumor to the roots of the tooth often make
them (roots) completely obscured, when seen in the radiograph.
• The lesion is surrounded by a thin zone of radiolucency at the periphery.
• Roots adjacent to the growing lesion often exhibit resorption of their apical third.
a highly mineralized tissue with intervening areas of loose, very cellular tissue with
increased vascularity.
• Multinucleated cells are present in large numbers in the central area and are
associated with active resorption.
• The root of the involved tooth may extend up to the center of the lesion and the
neoplastic cemental tissue is seen, continuation with the normal cementum of tooth.

DIFFERENTIAL DIAGNOSIS
• Osteoblastoma
• Osteoid osteoma
• Paget’s disease of bone
• Osteosarcoma
• Focal sclerosing osteomyelitis
TREATMENT: surgical excision
REFERENCES:
1. Textbook of oral and maxillofacial surgery 4th edition- Neelima anil malik
2.oral and maxillofacial surgery- fonseca 3rd edition (volume 2)
3.Odontogenic tumors and allied lesions- peter A. Reichert
4.oral and maxillofacial pathology - neville 4th edition
5.Oral and maxillofacial surgery Daniel M. Laskin (volume 2)
6. The World Health Organization Classification of Odontogenic Lesions: A
Summary of the Changes of the 2017 (4th) Edition

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