Odontogenic tumors

• Odontogenic tumors are neoplasms derived from the dental formative tissues.
Classification
A. Tumor of odontogenic epithelium
1- Ameloblastoma.
2- Calcifying epithelial odontogenic tumor.
3- Adenomatoid odontogenic tumor.
4- Squamous odontogenic tumor.
B. Tumors of odontogenic ectomesenchyme.
1- Odontogenic fibroma.
2- Cementifying fibroma.
3- Cementoblastoma.
4- Odontogenic myxoma.
C. Mixed odontogenic tumors
1- Ameloblastic fibroma.
2- Odontoma.
3- Ameloblastic fibro-odontome.
D. Malignant odontogenic tumors
1- Malignant ameloblastoma.
2- Ameloblastic carcinoma.
3- Ameloblastic fibrosarcoma.
4- Primary intra-osseous carcinoma
Tumors of odontogenic epithelium: They are composed only of odontogenic epithelium (enamel
organ and its derivatives) without any participation of odontogenic ectomesenchyme.
Tumors of odontogenic ectomesenchyme: They are composed principally of ectomesenchymal
elements (the dental papilla and the dental sac). Odontogenic epithelial remnants may be included
within these lesions but they don’t have any role in their pathogenesis.
Mixed odontogenic tumors: They are composed of odontogenic epithelial and ectomesenchymal
elements. Dental hard tissues may or may not be formed in these lesions i.e. differentiation of
odontogenic tissues within the lesion → inductive interactions → dental hard tissues.

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A- Tumors of odontogenic epithelium.
Ameloblastoma: It is the most common and important odontogenic epithelial tumor.
• Ameloblastoma is locally invasive (locally destructive) benign tumor.
Types
I- Conventional solid or multicystic → It is the most common subtype
II - Unicystic
III - Peripheral or extraosseous
I - Conventional (solid or multi-cystic) ameloblastoma
Clinical features
Age: The mean ages have been most commonly between 35 and 45 years.
Site: It may occur anywhere in the mandible or the maxilla (mandible > maxilla). About 85% of cases
occur in the mandible, most often in the posterior teeth bearing – ramus area.
Sign & symptoms
Early stage: It is slowly growing and asymptomatic so it may be discovered as an accidental finding.
As the tumor enlarges, the patient complains of a gradually increasing hard bony swelling → facial
deformity and expansion of the jaw bones.
Advanced cases: Egg shell crackling may be elicited due to thinning of the overlying bone.
Perforation of the bone and extension of the tumor into soft tissues are late features.
• Teeth in the area of the tumor may become loosened → tooth movement and malocclusion.
Radiographic features: The most typical radiographic feature is multilocular radioluceny. The lesion
is often described as having soap bubble appearance when the radiolucent loculations are large or
honey combed appearance when the loculations are small.
• It may appear radiographically as unilocular radiolucent defect with irregular scalloping border.
• Resorption of the roots of teeth adjacent to the tumor is common.
• In many cases, unerrupted tooth is associated with the radiolucent defect.
Histopathologic features: There is two main patterns, the follicular and plexiform depending on the
arrangement of the neoplastic epithelium.
1- Follicular pattern: It is the most commonly seen.
• The neoplastic epithelium arranged into islands or follicles, each resembles the enamel organ of the
developing tooth germ within fibrous connective tissue stroma.

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• Each follicle consists of: (1) central mass of loosely connected stellate reticulum like cells which are
angular cells resembling the stellate reticulum of the normal enamel organ (2) single layer of tall
columnar ameloblast- like cells surrounds this central core. The nuclei of ameloblast- like cells are
palisaded and located at the opposite pole of the basement membrane i.e. at the apical ends of cells.
2- Plexiform pattern
• The neoplastic epithelium is arranged into long anastomosing cords and strands; each of which shows
the same cell layers as for the follicular pattern. Thus, each strand is bounded by columnar ameloblast
like cells with palisaded and polarized nuclei, whilst the central area is occupied by stellate reticulum
like cells .The supporting stroma tends to be loosely arranged and vascular.
NB: A variety of changes can occur within the stellate reticulum like cells → other histopathological
patterns as:
Cystic ameloblastoma: Cystic formation is common in follicular type. Cystic degeneration of the
stellate reticulum like cells leads to cysts formation within the follicles.
Acanthomatous pattern: This term is applied when squamous metaplasia occurs in the central
portions of the epithelial follicles i.e. stellate reticulum like cells → squamoid cells. This is often
associated with keratin formation.
Granular cell pattern: The cytoplasm, usually of the stellate reticulum like cells becomes granular
eosinophilic cytoplasm.
Spindle cell pattern: It contains spindle cells in the central portion within the stellate area.
Basal cell pattern: Histopathologically, it resembles basal cell carcinoma of the skin.
II - Unicystic ameloblastoma
• It represents either cystic changes in a solid tumor or more probably ameloblastomatous changes in a
preexisting cyst (mostly, dentigerous cyst).
Clinical features
Age: It is often seen during the second to third decades of life (11-30 years).
Site: It occurs predominantly in the mandible, usually in the mandibular third molar area.
Signs & symptoms: Asymptomatic slowly growing hard bony swelling.
Radiographic features
• This lesion typically appears as a well defined unilocular radiolucency, usually associated with
unerupted tooth and is indistinguishable from a dentigerous cyst.

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Histopathologic features: The lesion presents as a cyst in which there is intraluminal, luminal or
mural growth of ameloblastoma i.e. three histologic variants of unicystic ameloblastoma.
Intra luminal variant: In this type one or more nodules of ameloblastoma projects from the cyst
lining into the lumen of the cyst.
Luminal variant: The tumor is confined to the luminal surface of the cyst. Proliferation of this typical
ameloblastomatous tissue leads to nodular thickening of the cyst lining.
Mural variant: Typical follicular or plexiform ameloblastoma infiltrates the fibrous wall of the cyst.
III - Peripheral (extra-osseous) ameloblastoma
Origin: This tumor probably arises from rests of dental lamina (Serre's rests) beneath the oral mucosa
Clinical features
Age: Mostly, it is seen in middle aged persons.
Sex: There is a slight predilection for males.
Site: They are most commonly found on the posterior gingival and alveolar mucosa. They are more
common in mandibular than in maxillary areas with a ratio of about 2:1.
Sign & symptoms: The clinical features are non specific, and most lesions are clinically considered to
represent a fibroma or pyogenic granuloma. It is soft, painless, sessile or pedunculated lesion with
smooth surface. Its color is pink or lighter (paler) than normal.
Radiographic features: Some cases exhibited erosion of the superficial alveolar bone → cup-shaped
erosion of buccal plate of bone.
Histopathologic features: It resembles the typical central ameloblastoma histologically.
Treatment: Surgical removal.
Calcifying epithelial odontogenic tumor (CEOT) (Pind borg tumor)
Clinical features
Age: It is most often encountered in middle age patients (mean age = 40 years).
Site: Mandible is affected twice as often as the maxilla (2:1). There is a predilection for the molar-
ramus region.
Signs & Symptoms: It is locally invasive but it appears to be less aggressive than the ameloblastoma.
• Painless, slowly growing hard bony swelling is the most common presenting sign.
▪ Lesions may be associated with impacted or unerrupted tooth.

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Radiographic features: It appears as a well-defined unilocular or multilocular radiolucency which
may contain radiopaque foci → driven snow appearance.
Histopathologic features
• The CEOT has discrete islands or sheets of polyhedral epithelial cells in a fibrous stroma.
• These polyhedral epithelial cells exhibit distinct outlines, prominent intercellular bridges and
abundant eosinophilic cytoplasm. They show variations in size, shape and number of nuclei i.e.
marked nuclear pleomorphism. Therefore, this tumor may be misdiagnosed as squamous cell
carcinoma. These cells could secrete amorphous, eosinophilic, hyalinized extracellular amyloid like
material.
NB: The precise nature of this amyloid like material is still uncertain. In general, this material stains as
amyloid i.e.
eosinophilic (H & E stain)
positive with congo red (orange to red)
apple green when viewed with polarized light after congo red stain
metachromatically with crystal violet.
Positive staining with thioflavine T (fluorescent method).
• Within this amyloid like material, concentric layers (rings) of calcifications may be developed →
Liesegang rings which are pathognomonic for pindborg tumor.
Treatment: Surgical removal
3. Adenomatoid odontogenic tumor (AOT)
• AOT is characterized by the formation of duct like or gland like structures (hence adenomatoid).
Clinical features
Age: AOT is seen in a narrow age range between 5-30 years, with most cases appearing in the second
decade (10-19 years).
Sex: Females are affected about twice as often as males (2:1).
Site: The majority of the tumors arise in the anterior portions of the jaws. It is found twice as often in
the maxilla as in the mandible (2:1), especially in the canine areas.
Signs & symptoms
• It is frequently asymptomatic and is discovered during routine radiographic examination.
• The lesion may be associated with unerupted tooth, mostly the canine.

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• Large lesions cause a painless expansion of the jaws → slowly enlarging swelling.
Radiographic features: These lesions revealed two different patterns:
Follicular pattern: The tumor appears as a well defined unilocular radiolucency that involves the
crown of unerupted tooth, most often a canine. This type of AOT can’t be differentiated
radiographically from the more common dentigerous cyst.
Extra follicular pattern: It is less often than the follicular type. The tumor appears as a well defined
unilocular radiolucency that is located between the roots of erupted teeth → may lead to their
divergence.
NB: The lesions (both types) are typically radiolucent but may have small opaque foci distributed
throughout reflecting the presence of dental hard deposits.
Histopathologic features: The histopathologic features of this lesion are distinctive and shouldn’t be
confused with any other odontogenic tumors.
• AOT is usually surrounded by a thick fibrous capsule i.e. well encapsulated lesion.
• The tumor consists of sheets, strands of polygonal or whorled masses of spindle-shaped epithelial
cells within fibrous tissue.
• These epithelial cells may form:
• Tubular or duct like structures which are the characteristic feature of AOT. These structures consist
of a central space surrounded by a single layer of columnar or cuboidal cells. The central space may be
empty or contain small amounts of eosinophilic material.
• Convoluted bands: Rosettes formed of 2 layers of tall columnar cells with no central space.
▪ Calcifications (enameloid, dentinoid or cementoid ) may be scattered throughout the tumor.
Treatment: Surgical removal
B- Tumors of odontogenic ectomesenchyme
Central odontogenic fibroma (COF)
• It is uncommon mesenchymal odontogenic tumor.
Clinical features
Age: It has been seen in all age groups.
Site: It is more common in the mandible than the maxilla (mandible > maxilla).
Signs & symptoms: Small odontogenic fibromas are usually asymptomatic. Meanwhile, large lesions
may be associated with localized bony expansion or loosening of teeth.

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Radiographic features
• Small lesions tend to be well defined unilocular, radiolucent lesions.
• Large lesions tend to be multilocular radiolucencies.
Histopathologic features: Two patterns are generally ascribed:
Simple type: The tumor mass is made of scattered fibroblasts with fine collagen fibers in the
background. Small minimal foci of odontogenic epithelial rests may or may not be present. Occasional
foci of dystrophic calcification may be present.
World Health Organization (WHO) type: This type has a more complex pattern, which often
consists of relatively mature fibrous connective tissue with the collagen fibers arranged in interlacing
bundles. Odontogenic epithelial rests and calcifications are prominent throughout the lesion.
Treatment: Surgical removal
Odontogenic myxoma
• Odontogenic myxoma is locally invasive benign neoplasm.
Clinical features
Age: Myxomas are predominantly seen in young adults.
Site: The mandible is involved more commonly than maxilla.
Signs & Symptoms: Small lesions are asymptomatic and discovered during radiographic examination.
• Large lesions are associated with painless expansion of the involved bone.
Radiographic features
• The myxoma appears as well circumscribed unilocular or multilocular radiolucency that may
displace or cause resorption of teeth in the area of the tumor. It is often multilocular, frequently with a
honey comb pattern while large myxomas may show a soap bubble radiolucent pattern.
Histopathologic features
• Odontogenic myxomas bear a close microscopic resemblance to the mesenchymal portion of a
developing tooth.
• The tumor consists of stellate cells with long anastomosing processes in an abundant, loose mucoid
stroma.
• Small islands of inactive odontogenic epithelial rests may be scattered throughout the myxoid
background.

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Treatment: Surgical excision is the treatment of choice. Recurrence rate is about 25%. This could be
attributed to their infiltrative behavior and absence of capsule.
The cementoma group
• They are lesions containing cementum or cementum-like tissue. They are a complex group with
different characteristics. Some are probably true neoplasms but others may represent dysplastic or
reactive processes.
Benign cementoblastoma (true cementoma)
• It is a true neoplasm of functional cementoblasts which forms a large mass of cementum or
cementum like tissue on the tooth root.
Clinical features
Age: It occurs in second and third decades of life, typically before 25 years.
Site: It is more often seen in the mandible than in the maxilla (greater than 75% arise in the mandible)
i.e. mandible > maxilla (3:1). It is more common in the molar and premolar regions (90% of cases).
• Most cases involve the mandibular first permanent molar. They are attached to the root.
Sign & Symptoms
Low grade intermitted pain present in about 2/3
Slowly enlarging swelling of reported patients.
It is intimately associated with the root of the tooth, and the tooth remains vital.
Radiographic features
• Well demarcated mottled or dense radiopaque mass with a thin radiolucent rim attached to the tooth
root. Initially, it appears as well defined periapical radiolucency.
Histological features
• The tumor consists of a cementum like material with numerous criss - crossing reversal lines and
containing scattered cells lying in lacunae.
• These mineralized masses are frequently lined by hyperchromatic cementoblasts.
• Cellular fibro-vascular tissue is present between the mineralized trabeculae.
Treatment
• Surgical excision of the mass with root amputation and endodontic treatment of the involved tooth
may be considered.

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• Surgical extraction of the tooth together with the attached calcified mass (if not indicated for
endodontic treatment).
NB: Incomplete removal of the tumor → progressive growth of the tumor after extraction of the
involved tooth.
Periapical cemental dysplasia
• This lesion represents a reactive or dysplastic process rather than a neoplastic one. It appears to be an
unusual response of periapical bone and cementum to some local factors.
• The etiology is unknown, chronic inflammation is not implicated and the involved teeth remain vital.
Clinical features
Age: It appears in middle age (around 40 years; mostly, postmenopausal females)).
Sex: Women, especially black women are affected more than are men (10:1).
Site: This condition arises most frequently in the incisor region of the mandible.
Sign & Symptoms: It is usually symptomless and is discovered accidentally. It doesn’t produce jaw
expansion i.e. no swelling.
Radiographic features
The radiographic appearance varies with the stage of development of the lesion:
(A) Initial phase (osteolytic or resorptive stage): appears as a periapical radiolucency.
(B) The second phase (mixed stage) (cementoblastic stage): mixed or mottled pattern.
(C) The final phase (mature stage): well defined radiopacity surrounded by a thin, lucent ring.
NB: Teeth exhibiting periapical cemental dysplasia are vital.
Histopathologic features
The histological appearance varies with the stage of development of the lesion:
(A) Initial phase (osteolytic phase): loss of bony substance and replacement by connective tissue.
(B) Mixed phase: There is a mixture of benign fibrous tissue, bone and cementum.
(C) The final phase: cementoblastic and osteoclastic activities → increased density and sizes of the
calcified tissues deposited within the fibrous tissue.
NB: Age, gender, location, radiographic appearance and tooth vitality are considered together as
diagnostic features of this condition.
Treatment: No treatment is required for this condition as once the opaque stage is reached; the lesion
stabilized and causes no complications. Teeth remain vital throughout the entire process.

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Cementifying fibroma
• Cementifying fibroma may be impossible to separate from ossifying fibroma. Based on the marked
similarity between the two regarding predilection for age of occurrence, sex, race, location,
radiographic appearance and clinical behavior, these two lesions represent the same basic neoplastic
process.
• The only feature that has served to separate the two is the microscopic identification of cementum (in
cementifying fibroma) or bone (in ossifying fibroma).
Clinical features
Age: It occurs chiefly in adults around age of 40 years i.e. middle age.
Sex: There is a marked predilection for occurrence in females (2:1).
Site: The lesion is more often seen in the mandible than in maxilla (molar & premolar area).
Sign & symptoms: Asymptomatic slowly growing swelling.
Radiographic features
• This neoplasm presents a variable radiographic appearance depending upon its stage of development.
It may appear as a well circumscribed:
Lucent lesion (early stage)
Lucent with opaque foci (mixed stage) or
Opaque with a thin peripheral radiolucent rim representing the fibrous capsule.
Histopathologic features
• The lesion is composed basically of fibrous connective tissue with small foci of basophilic masses of
cementum like tissue.
• The lesion is well encapsulated lesion (fibrous capsule).
C- Mixed odontogenic tumors
Ameloblastic Fibroma
Clinical features
Age: This neoplasm occurs predominantly in children and young adults (first two decades of life). The
mean age is about 12 years.
Sex: This tumor is slightly more common in males than in females.
Site: Although any region may be affected, the mandibular molar ramus area is the most common site.
Signs & symptoms: Small lesions are asymptomatic.

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• Large tumors present as slowly enlarging painless swelling.
Radiographic features: It appears as well-defined unilocular or multilocular radiolucency. An
unerupted tooth may be associated with the lesion.
Histopathologic features: This lesion is usually surrounded by a fibrous capsule. The tumor mass is
composed of:
Mesenchymal component:
• It is made up of a highly cellular primitive connective tissue resembling the primitive dental papilla
of the tooth germ. It consists of stellate and ovoid cells in a loose matrix with inconspicuous collagen
formation.
Epithelial component
• This component has one of two patterns, both of which are usually present in any given case.
• The most common epithelial pattern consists of ribbons, strands, cords or enamel organ like
structures of odontogenic epithelium. These cords are typically two cells wide of cuboidal or columnar
cells.
• The other epithelial pattern consists of small discrete islands, nests, rosettes or follicles of
odontogenic epithelium. This epithelial component shows a peripheral layer of tall columnar or
cuboidal cells (ameloblast- like cells) which encloses a few stellate reticulum like cells.
Treatment: Because of tumor encapsulation and the general lack of invasive capacity, this lesion is
treated with conservative surgical procedures as curettage or excision.
Ameloblastic fibro-odontoma (AFO)
• It is defined as a tumor with the general features of an ameloblastic fibroma but also contains enamel
and dentine.
Odontoma (Odontome)
• They are known as mixed odontogenic tumors.
• Odontomas are considered to be developmental anomalies (hamartomas) rather than neoplasms.
NB: Hamartoma is a congenital malformation that presents as a mass of cells and tissue not foreign to
the organ in which it occurs. It stops growth with growth of the body.
Types: Odontomas are subdivided into:
Compound odontoma: They appear as numerous small tooth like structures i.e. denticles or
rudimentary teeth.

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Complex odontoma: They appear as irregular masses or conglomerations of hard tissues which bears
no anatomic resemblance to a tooth.
Clinical features
Age: Most odontomas are detected during the first two decades of life.
Site: The maxilla is affected slightly more often than the mandible. Although compound and complex
odontomas may be found in any site, the compound type is more often seen in the anterior maxilla
while the complex type occurs more often in the molar regions of either jaw.
Signs & symptoms
• The majority of these lesions are completely asymptomatic, being discovered on a routine
radiographic examination. Large odontomas cause expansion → jaw swelling.
• Impacted permanent tooth and retained deciduous tooth are clinical signs that may reflect the
presence of an odontoma.
Radiographic features
• The compound odontoma appears as a collection of several (may be tens or dozens) teeth like
structures of varying sizes and shapes in a single focus surrounded by a narrow radiolucent rim.
• The complex odontoma appears as radiopaque mass with irregular outlines which is surrounded by a
thin radiolucent rim.
• Odontomas are frequently associated with unerrupted tooth.
Histopathologic features
The compound odontoma consists of multiple, small teeth like structures contained in a loose fibrous
stroma.
• Normal appearing enamel, dentine, cementum and pulp may be seen in these lesions. They exhibit a
normal relation to one another i.e. the same arrangement within tooth (enamel, then dentine then pulp
or cementum, then dentine then pulp).
• The mature enamel caps of the teeth like structures are lost during decalcification for preparation of
the microscopic section → enamel space.
The complex odontoma consists of diffuse masses of dentine. This dentine encloses clefts or hollow
circular structures that contained the enamel that was removed during decalcification i.e. It lacks
normal arrangement of dental hard tissues (enamel & dentine & pulp don’t exhibit the normal relation
to one another).

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Treatment: Odontomas are treated by simple local excision, and the prognosis is excellent.
D. Malignant odontogenic tumors
Primary intra-osseous carcinoma
• It is squamous cell carcinoma arising within the jaw, having no initial connection with the oral
mucosa.
Origin: It arises from odontogenic epithelium.
Radiographic features: Bone lysis can be detected on radiographs as moth-eaten radiolucency.
• It may be suggested clinically by mobility of teeth, painless rapidly growing hard bony swelling,
numbness & parathesia, ulceration of overlying mucosa or skin and pathologic fracture.
Malignant behavior of ameloblastoma
• Rarely, ameloblastoma exhibits frank malignant behavior with development of metastases.
Origin: These tumors may arise de novo or from a pre-existing benign odontogenic tumors or cysts
i.e. ex-ameloblastoma or ex-odontogenic cyst.
Malignant ameloblastomatous lesions have been divided into two subtypes:
Malignant ameloblastoma, in which the primary and metastatic lesions are microscopically well
differentiated i.e. the primary jaw tumor and the metastatic deposits show microscopic features similar
to those of benign ameloblastomas.
Ameloblastic carcinoma, in which the epithelial component of the lesions
(primary and metastatic) exhibits cytologic features of malignancy i.e. primary and metastatic tumor
are poorly differentiated.
• Metastases from ameloblastomas are most often found in the lung (75% of the cases).
• Cervical lymph nodes are the second most common site.
Radiographic features
• Malignant ameloblastomas can’t be distinguished from their benign counterparts.
• Ameloblastic carcinomas present as ill defined radiolucencies.
Ameloblastic fibrosarcoma
• The ameloblastic fibrosarcoma is the malignant counter part of the ameloblastic fibroma in which the
mesenchymal element (fibroblasts) are malignant meanwhile the epithelial component remains benign.

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