Odontogenic Tumors

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ODONTOGENIC TUMORS

Dr. Madhan Prabhu, OMFS PG


Contents
Introduction

Classification

Diagnostic Criteria

Features

Management

Recent Advances

References
Introduction
Tumor or neoplasm is an abnormal new, uncoordinated growth in the body, which results from excessive, autonomous, purposeless
proliferation of cells, which continues its growth, even after cessation of stimuli

Tumors are broadly divided into two groups Benign and Malignant, depending upon their behaviour pattern and cellular structure.

A benign tumor is a mass of cells, tissues/organs resembling those normally present, but arranged atypically and behave abnormally (The term
benign implies mild and non-progressive). It grows slowly and is usually encapsulated and it enlarges by peripheral expansion, pushes away the
adjoining structures and exhibits no metastasis, however, it may be locally aggressive.

A malignant tumor, rapidly infiltrates the surrounding tissues, including vital structures and endangers the life of its host. It also shows
metastasis in the distant parts of the body usually through lymph and blood streams. The malignant tumors may arise as de novo, or often from
their benign precursor.
Introduction
According to WHO - “Odontogenic tumours and tumour-like lesions constitute a group of heterogeneous diseases that range from hamartomatous or
non-neoplastic tissue proliferations to benign neoplasms and finally malignant tumours with metastatic potential. They are derived from epithelial,
ectomesenchymal and/or mesenchymal elements of the tooth-forming apparatus. Odontogenic tumours are rare, some being extremely rare, but can
pose a significant diagnostic and therapeutic challenge”

The tumours are sub - classified into Epithelial origin, Mixed origin and Mesenchymal origin

In 2022 - The only newly defined entity in odontogenic lesions is adenoid ameloblastoma, which is classified under benign epithelial odontogenic
tumors.

A very important change was made that added ‘essential and desirable diagnostic criteria’ for each entity to highlight the features considered
indispensable for diagnosis.
Ectodermal vs Mesenchymal

Ectodermal — epithelial origin

Dental Lamina - its remnants - Epithelial rests of Serres

Enamel organ - reduced enamel epithelium

Hertwig’s epithelial Root Sheath (HERS)

Mesenchymal - connective tissue origin

Dental Papilla

Dental Sac
Classification
The World Health Organization Classification of Odontogenic Lesions - 2022

Benign mesenchymal odontogenic tumours


Benign epithelial odontogenic tumours
Odontogenic fibroma
Adenomatoid odontogenic tumour
Cementoblastoma
Squamous odontogenic tumour

Calcifying epithelial odontogenic tumour Cemento-ossifying fibroma

Ameloblastoma, unicystic Odontogenic myxoma

Ameloblastoma, extra osseous/peripheral


Malignant odontogenic tumours
Ameloblastoma, conventional
Sclerosing odontogenic carcinoma
Adenoid ameloblastoma
Ameloblastic carcinoma
Metastasising ameloblastoma

Benign mixed epithelial Clear cell odontogenic carcinoma

Odontoma Ghost cell odontogenic carcinoma

Primordial odontogenic tumour Primary intraosseous carcinoma

Ameloblastic fibroma
Odontogenic carcinosarcoma
Dentinogenic ghost cell tumour
Odontogenic sarcomas
Diagnostic Criteria

There is always a possibility of odontogenic lesions developing from these tissues; resulting in the development of malformations,
hamartomas and neoplasms.

A malformation: It is not neoplastic, but it can cause a functional or aesthetic problem, because of its size or anatomical site.

A hamartoma: It is a benign lesion composed of a new overgrowth of mature cells on existing blood vessels. (A lesion resulting from
faulty development of the embryo).

Odontogenic tumors: A group of neoplasm or tumor like malformations arising from cells of odontogenic apparatus or their remnants.
The abnormal tissue in each of these tumors often can be correlated with similar tissue in normal odontogenesis. They are slow-growing
and tend to spread by direct extension and not by metastasis.
Diagnostic Criteria
Soluk-Tekkesin M, Wright JM. The World Health Organization Classification of Odontogenic Lesions: A Summary of the Changes of the 2022 (5th) Edition. Turk Patoloji Derg. 2022;38(2):168-184. doi: 10.5146/tjpath.2022.01573. PMID: 35578902; PMCID: PMC9999699.

Odontogenic Tumors Age/Gender/Localization Essential Diagnostic Criteria

- Site in alveolar processes of jaws


- 2nd-3rd decades/Female - Epithelial nodular structure
Adenomatoid odontogenic tumor - Anterior maxilla - Rosettes of spindled to columnar epithelial cells
- Pericoronal - Duct-like structures
- Minimal stroma

- Mean age at diagnosis is 34.8 - Site in tooth bearing areas of jaw


- No gender predilection - Closely packed islands of cytologically bland epithelium
Squamous odontogenic tumor - Uniform squamous differentiation without significant keratinization
- Anterior maxilla and posterior mandible - No peripheral palisading and stellate reticulum

- 4th decade -Tooth-bearing areas of the jaws


- No gender predilection - Sheets, islands and cords of polyhedral cells with distinct cell borders
Calcifying epithelial odontogenic tumor - Very few or no mitoses
- Body of the mandible - Amyloid present

- 2nd decade - Single cyst


Ameloblastoma, unicystic - Slightly male - Ameloblastoma-like epithelial lining
- Posterior body of mandible and ramus
Diagnostic Criteria
- 5th-7th decades - Site in gingiva or edentulous alveolar mucosa
Ameloblastoma, extraosseous - Slightly male - No intraosseous component
- Soft tissue of mandibular premolar and maxillary molar regions - Histopathologic features as conventional ameloblastoma

- Islands/strands of odontogenic epithelium bounded by cuboidal/columnar cells with palisaded,


- 4th-5th decades
hyperchromatic nuclei
Ameloblastoma, conventional - No gender predilection
- Reverse polarity
- Posterior molar site of mandible
- Loose central epithelium resembling stellate reticulum

- 4th decade - Ameloblastoma-like component; duct-like structures


Adenoid ameloblastoma - Slightly male - Whorls/morules
- No site predilection - Cribriform architecture

- A mean age 45 years


Both in primary tumor and metastatic tumor:
- Slightly male
Metastasizing ameloblastoma - Benign conventional ameloblastoma
- Primary tumor site: mandible
- No cytological atypia or features of malignancy
- Metastatic site: lung

- 2nd-3rd decades Cx:


Odontoma
- No gender predilection - Conglomerate mass of enamel and dentin
-Complex
- Posterior body of the mandible for Cx Cd:
-Compound
- Anterior maxilla for Cd - Multiple, small tooth-like structures
Diagnostic Criteria
- 1st-2nd decades
- Mass of myxoid dental papilla-like tissue
Primordial odontogenic tumor - Slightly male
- Entire periphery covered by columnar or cuboidal enamel epithelium
- Posterior mandible

- 1st-2nd decades - A well-defined and corticated radiolucency


Ameloblastic fibroma - Slightly male - Bland hypercellular, dental papilla-like mesenchyme
- Posterior mandible - Dispersed bilaminar strands of cuboidal or columnar odontogenic epithelium

- 3rd- 5th decades - Solid tumor


- Male - Conventional ameloblastoma-like epithelium
Dentinogenic ghost cell tumor
- Almost equally in the maxilla and mandible (posterior regions in - Ghost cells
both jaws) - Dentinoid

- Site in tooth bearing segments of the jaws


- A mean age of 34 years
- A well-defined lesion radiologically
Odontogenic fibroma - Female
- Bland fibrous connective tissue of varying cellularity
- Slightly maxilla (anterior to the first molar)
- Varying amounts of odontogenic epithelium

- Mass fused to a tooth root


- 2nd-3rd decades - Densely mineralized
Cementoblastoma - No gender predilection - Radiating peripheral matrix
- Posterior mandible (the apical third of permanent first molar) - Plump cementoblasts
- No fibro-osseous component
Diagnostic Criteria
- 3rd -4th decades - Site in tooth bearing region of jaws
Cemento-ossifying fibroma
- Female - Benign fibro-osseous histology
- Premolar and molar region of mandible - Well demarcated

- 2nd-3rd decades - Site in tooth-bearing segments of jaws


Odontogenic myxoma
- Female - Myxoid stroma with variable collagenization
- Premolar or molar region of mandible - Sparse stellate or spindle shaped cells

- 5th-7th decades - A poorly defined radiolucency


Sclerosing odontogenic carcinoma - Slightly female - Thin cords and nests of epithelium
- Posterior mandible - A dense, fibrocollagenous sclerotic stroma

- A median age of 49 years - A poorly defined lesion radiologically


Ameloblastic carcinoma
- Male - Histological resemblance to ameloblastoma with cytological atypia
- Posterior mandible - Features of malignancy

- Site in jaws and ill-defined radiolucency


- A mean age: 53 years
- Prominent clear cell phenotype
Clear cell odontogenic carcinoma - Female
- Infiltrative margin
- Mandible (posterior body-lower ramus)
- Exclusion of metastatic disease
Diagnostic Criteria
- Poorly demarcated lesion radiologically
- 4th-7th decades
- Ameloblastoma-like epithelium
Ghost cell odontogenic carcinoma - Male
- Prominent ghost cells
- Maxilla
- Cytological evidence of malignancy

- The mean age: 55-60 years - Destructive central jaw lesion


Primary intraosseous carcinoma, NOS - Male - Absence of a communication with the surface mucosa or antrum
- Mandible (posterior body and ramus) - Exclusion of metastatic disease

- Poorly demarcated lesion in tooth bearing segment


- No age incidence
- Carcinoma and sarcoma components
Odontogenic carcinosarcoma - Male
- Significant cytologic atypia in both components
- Posterior mandible
- Exclusion of spindle cell carcinoma.

- Origin in tooth bearing segment of jaws


- The average age: upper 3rd decade
- Mixed odontogenic neoplasm
Odontogenic sarcomas - Male
- Cytologically bland epithelial component
- Posterior mandible
- Cytologically malignant ectomesenchymal component
Odontogenic Tumors

Benign Epithelial Odontogenic Tumor

Tumors that are composed of Odontogenic epithelium, Mature connective tissue, Absence of odontogenic ectomesenchyme
Features - Ameloblastoma
Other names

Adamantinoma

Adamantoblastoma

Second most common odontogenic tumor

Ameloblastoma is the tumor of odontogenic epithelial origin. It may arise from

Rests of dental lamina

Developing enamel organ

Epithelial lining of an odontogenic cyst

Basal cells of oral mucosa


Ameloblastoma
It is typically slow-growing, locally invasive and runs a benign course.

H.G.B. Robinson described it as being tumor that is


"Usually unicentric, non-functional, intermittent in growth, anatomically benign and clinically persistent.”

Ameloblastomas occur in 3 different clinico-radiographic situations requiring different therapeutic considerations and having different
prognosis.

Conventional Solid/Multi-cystic (86 % of all cases)

Unicystic (13 % of all cases)

Peripheral or Extra-osseous (1 % of all cases)


Ameloblastoma
Staging of Ameloblastoma Yang et al.

Stage I: Maximum Tumor size less than 6 cms

Stage II: Maximum Tumor size more than 6 cms or invading the Maxillary sinus or orbital floor

Stage III: Tumor invasion of the skull base or metastasis into regional lymph nodes

There is a significant relationship between time to recurrence and tumor stage, with the earliest recurrence in stage III tumors.
Ameloblastoma
Classification according to radiological Features

Solid / Multicystic

Unicystic

Extra-osseous / peripheral
Solid / Multicystic Ameloblastoma

Clinical Features

Patient Age: 3rd to 7th decades.

Sex Predilection: Approximately equal.

Location: 80 % in mandible; 70 % in posterior regions.


Solid / Multicystic Ameloblastoma

Greater frequency in blacks

Pain less swelling or expansion of jaw


Solid / Multicystic Ameloblastoma

Radiographic Appearance:

Radiolucent lesion - well-circumscribed;

Unilocular or Multilocular (soap-bubble, honeycomb)

Buccal and lingual expansion of cortical plates

Resorption of roots of teeth adjacent to tumor


Solid / Multicystic Ameloblastoma

Histological Feature:

Plexiform

Follicular

Acanthomatous

Granular

Desmoplastic

Basal Cell

Other Variants - Clear cell, Papilliferous keratoameloblastoma, Mucous


Solid / Multicystic Ameloblastoma

The follicular type is composed of islands of epithelium which resemble the enamel organ in a mature fibrous connective tissue stoma.

The Acanthomatous type shows evidence of extensive squamous metaplasia with keratin formation in the island of odontogenic epithelium.
Solid / Multicystic Ameloblastoma

In the granular cell type there is transformation of groups of epithelial cells to granular cells;

Common in young patients

Clinically aggressive.
Solid / Multicystic Ameloblastoma

The desmoplastic - islands/cords of odontogenic epithelium in a very dense collagenous stroma.

Predilection for the anterior maxilla

May appear as a radiolucent-radiopaque lesion.


Solid / Multicystic Ameloblastoma

The basal cell type is the least common and is composed of uniform basaloid cells with no stellate reticulum.
Solid / Multicystic Ameloblastoma

The plexiform type is composed of long, anastomosing cords or larger sheets of odontogenic epithelium.

Its stroma tends to be loose and more vascular.


Solid / Multicystic Ameloblastoma

Treatment

Simple enucleation

Curettage

En bloc resection.

Marginal resection - most widely used method of treatment with the least recurrences reported (up to 15 %).

Most surgeons advocate a margin of at least 1.0 cm beyond the radiographic limits of the tumor as the tumor often extends beyond the
apparent radiologic/clinical margins.
Solid / Multicystic Ameloblastoma

Prognosis

Curettage has resulted in recurrence rates ranging from 55-90 %.

Marginal resection has resulted in approximately a 15 % recurrence rate.

Ameloblastomas arising in the maxilla are particularly dangerous as it is often difficult in getting adequate margins.
Unicystic Ameloblastoma

Clinical Features

Patient Age: younger than those with the solid/multicystic


form.

50% are diagnosed during the second decade of life.

Sex Predilection: Same as for the SMA

Location:
90% occur in the mandible usually in the
posterior region.
Unicystic Ameloblastoma
Radiographic Appearance

Typically appears as a radiolucency around the crown of an unerupted tooth (most commonly a mandibular third molar).
Unicystic Ameloblastoma
Histologic Features

Fibrous cystic wall

Lining epithelium

Basal columnar or cuboidal cells with resembling ameloblast like cells

Hyperchromatic nucleus with reversal of polarity

Cytoplasmic vacuolization

Nuclear palisading

Overlying epithelial cells are loosely cohesive resembling stellate reticulum


Unicystic Ameloblastoma

Three histopathologic variants are recognized:

Luminal: the tumor is confined to the luminal surface of the cyst.

Intraluminal/plexiform: the tumor projects from the cystic lining; sometimes resembles the plexiform type of solid/multicystic
ameloblastoma.

Mural: the tumor infiltrates the fibrous cystic wall.


Unicystic Ameloblastoma

Treatment and Prognosis

Enucleation of the cyst is probably adequate for the luminal and intraluminal/plexiform types.

Treatment of the mural type is controversial with some surgeons believing that local resection is best.

10-20 % recurrence after enucleation and curettage with all unicystic ameloblastomas.
Peripheral Ameloblastoma

These tumors are extra-osseous and therefore occupy the lamina propria underneath the surface epithelium but outside of the bone.

Histologically, these lesions have the same features as the intraosseous forms of the tumor.
Peripheral Ameloblastoma
Clinical Features

Patient Age:
Wide age range but most occur during
middle-age.

Gender Predilection: This is not known.

Location: Posterior gingival/alveolar mucosa is involved most


frequently.

There is a slight predilection for the mandible.


Peripheral Ameloblastoma

Radiographic Appearance:

Although not in bone, a few cases have shown superficial erosion of the alvelolar bone.

Histologic Appearance:

Islands of ameloblastic epithelium are observed in the lamina propria; plexiform and follicular patterns are the most common; in 50% of the
cases the tumor connects with the basal cell layer of the surface epithelium.
Peripheral Ameloblastoma

Treatment and Prognosis

Unlike its intraosseous counterpart, this tumor has an innocuous clinical behaviour.

Patients respond well to local surgical excision.

Some reports indicate a 25 % recurrence rate but in these cases as second surgical procedure results in cure.

There has been a rare malignant change reported.


Adenomatoid Odontogenic Tumour

Formerly called an adenoameloblastoma

AOT represents about 3-7% of all odontogenic tumors.

This epithelial tumor has an inductive effect on the odontogenic ectomesenchyme with dentinoid frequently being produced.
Adenomatoid Odontogenic Tumour

Tumor classified as hemartoma

Tumor cells are derived from

Enamel organ epithelium

Remnants of dental lamina

AOT is called as 2/3rd tumor

2/3rd cases occurs in age group pf 10-19 yrs

2/3rd cases seen in females

2/3rd in the anterior jaws

2/3rd in maxilla
Adenomatoid Odontogenic Tumour
Clinical Features

Patient Age: second decade with a mean around 17 years.

Gender Predilection: Females, 2:1.

Location:
65% of them occur in the maxilla with 65%
occurring in the canine region. 75% of the cases are associated with the crown
of an unerupted tooth. On rare occasion the lesion is extraosseous.

Frequently asymtomatic

Painless expansion
Adenomatoid Odontogenic Tumour
These lesions are frequently asymptomatic and therefore are
discovered upon routine radiographic examination. AOTs may also
block the eruption of a permanent tooth and be discovered when
radiographs are taken to "search for" the unerupted tooth.
Adenomatoid Odontogenic Tumour
Radiographic and Additional Features

AOTs typically appear as pericoronal radiolucencies, which may have radiopaque material («snowflake" calcifications) within the lucency.
Adenomatoid Odontogenic Tumour
Adenomatoid Odontogenic Tumour

Histologic Features

The lesion is usually surrounded by a thick, fibrous capsule.

The tumor is composed of spindle-shaped epithelial cells that form sheets, strands or whorled masses with little connective tissue.

The epithelial cells may form rosette-like structures, tubular or duct-like structures may be prominent or absent.

Calcifications may be observed in the tumor mass.


Adenomatoid Odontogenic Tumour
Treatment and Prognosis

Enucleation is the treatment of choice as the tumor is easily removed from the bone.

AOTs seldom recur.


Calcifying Epithelial Odontogenic Tumor
Pindborg Tumor

Pindborg tumor accounts for < 1 % of all odontogenic


tumors.

It is clearly of odontogenic origin but its histogenesis is uncertain.

The tumor cells are said to resemble

Stratum intermedium.

Enamel organ

Dental lamina
Calcifying Epithelial Odontogenic Tumor
Clinical Features

Patient Age: 2nd to 10th decades (mean age 40 years).

Gender Predilection: no reported sex predilection.

Location: 75 % of the CEOs occur in the mandible with most occurring in the posterior region.

Painless swelling slow growing

Peripheral - sessile gingival mass

Anterior gingiva
Calcifying Epithelial Odontogenic Tumor
Bony lesions most commonly present as painless, slow-growing swellings.

Peripheral lesions typically appear as non-specific sessile gingival masses.

Radiographic Features

CEOTs occur as radiolucent lesions with/without opaque foci.

They are usually well-circumscribed and may be


unilocular or multilocular.

Slightly over 50% of the CEOTs are associated with an unerupted tooth.
Calcifying Epithelial Odontogenic Tumor
Calcifying Epithelial Odontogenic Tumor
Histologic Features

This lesion is typically composed of islands, sheets or strands of polyhedral epithelial cells in a fibrous stroma.

Areas of amorphous, eosinophilic, hyalinized extracellular material may be scattered throughout.

Cells outlines are distinct and intercellular bridges may be seen.

Nuclei show considerable variation with giant nuclei and pleomorphism observed.

Calcifications may be noted as well as amyloid-like material. Liesegang rings also may be present.
Calcifying Epithelial Odontogenic Tumor
Treatment and Prognosis

Conservative local resection is the treatment of choice as these lesions are typically less aggressive than the ameloblastoma.

With this treatment the recurrence rate is approximately 15 % and the overall prognosis is good.
Squamous Odontogenic Tumor
Clinical Features

Patient Age: Second through the seventh decades (mean


40 years).

Gender Predilection: None

Location: SOTs occur with about equal frequency in maxilla and mandible. They are more common in the anterior regions of the jaws than in
the posterior. The lesions occur in the alveolar process.
Squamous Odontogenic Tumor
Radiographic Features

SOTs appear as non-specific radiolucent lesions. They may be well-circumscribed or ill-defined. They often appear triangular in shape and
lateral to the tooth root.
Squamous Odontogenic Tumor
Histologic Features

Islands of bland-appearing squamous epithelium

Mature fibrous connective tissue stroma.

The peripheral cells do not show the characteristic polarization seen in the ameloblastoma.
Squamous Odontogenic Tumor
Treatment and Prognosis

SOTs often present as painless gingival swellings associated with tooth mobility. Approximately 25 % are asymptomatic.

Conservative local excision or curettage appears to be effective treatment and there have only be a few recurrences reported.
Odontogenic Tumors

Benign mixed epithelial & mesenchymal


odontogenic tumours

This type of tumors is composed of proliferating odontogenic epithelium in a cellular ectomesenchyme resembling the dental papilla
Ameloblastic Fibroma
Clinical Features

This true mixed odontogenic tumor is more common in patients


in the first and second decades of life with a mean of 14 years.

It is slightly more common in males than females.

Approximately 70 % of the ameloblastic fibromas occur in the


posterior mandible.
Ameloblastic Fibroma
Clinical Features

This true mixed odontogenic tumor is more common in patients in the first and second decades of life with a mean of 14 years.

It is slightly more common in males than females.

Approximately 70 % of the ameloblastic fibromas occur in the posterior mandible.


Ameloblastic Fibroma
Radiographic Features

Generally, these lesions appear as either a unilocular or multilocular radiolucency.

They tend to be well-defined and may have a sclerotic border.

Approximately, 50 % are associated with an unerupted tooth.


Ameloblastic Fibroma
Ameloblastic Fibroma
Histologic Features

The tumor is composed of a cell-rich mesenchymal tissue resembling the primitive dental papilla admixed with proliferating odontogenic
epithelium.
Ameloblastic Fibroma
Additional Features, Treatment and Prognosis

• The tumor is often encapsulated with small tumors usually being asymptomatic.

Larger tumors produce swelling,

which can expand the cortex and be quite pronounced.

Most ameloblastic fibromas are treated by conservative surgical excision; however, a 20 % recurrence rate has led some surgeons to
recommend a more aggressive approach.
Odontoma
The odontoma is the most common odontogenic tumor.

It is not a true neoplasm but rather is considered to be a developmental anomaly (hamartoma).

Two types of odontomas are recognized:

Compound: this type of odontoma is composed of multiple small tooth-like structures.

Complex: this lesion is composed of a conglomerate mass of enamel and dentin, which bears no anatomic resemblance to a tooth.
Odontoma
Clinical Features

Patient Age: Most cases are recognized during the second decade of life with a mean of 14 years.

Gender Predilection: Approximately equal.

Location: Some what more common in the maxilla. The compound type is more often in the anterior maxilla while the complex type occurs
more often in the posterior regions of either jaw.
Odontoma
Radiographic Features

Early lesions are radiolucent with smooth, well-defined contours.


Later a well-defined radiopaque appearance develops.

The compound type shows apparent tooth shapes while the complex type appears as a uniform opaque mass with no apparent tooth shapes
present.
Odontoma
Odontoma
Additional Features

Most odontomas are small and do not exceed the size of a normal tooth in the region.

However, large ones do occur and these may cause expansion of the jaw.

Most odontomas are asymptomatic and as a result are discovered upon routine radiographic examination.

Odontomas may block the eruption of a permanent tooth and in these cases are often discovered when "searching for" the "missing" tooth
radiographically.
Odontoma
Histologic Features

The compound odontoma is composed of enamel, dentin and cementum arrange in recognizable tooth forms; some enamel matrix may be
retained in immature and hypomineralized specimens.

The complex odontoma is composed of enamel, dentin and cementum but these tissues are arranged in a random manner that bears no
morphological resemblance to a tooth.
Odontoma
Treatment and Prognosis

Odontomas are treated by simple local excision and the prognosis is excellent.
Odontogenic Tumors

Tumours of Odontogenic Ectomesenchyme

Benign mesenchymal odontogenic tumors are lesions derived from the mesenchymal components of the tooth-forming apparatus and are
consequently found within the jawbone
Odontogenic Fibroma
Clinical Features

Fewer than 70 cases have been reported in the English literature.

Patient Age: Age from 9-80 years old with a mean of 40 years.

Gender Predilection: Females, 7.4:1 in one study.

Location: 60% occur in the maxilla - located anterior to the 15 molar.

Mandible - approximately 50 % occur in the posterior jaw.


Odontogenic Fibroma
Odontogenic Fibroma
Radiographic Appearance

The odontogenic fibroma

Well-defined

Unilocular radiolucency

It is often associated with the apical area of an erupted tooth.

Larger lesions - multilocular

Many odontogenic fibromas have sclerotic borders.

Root resorption is common.


Odontogenic Fibroma
Odontogenic Fibroma
Additional Features

Small odontogenic fibromas are usually asymptomatic.

The larger lesions may be associated with localized bony expansion of the jaw or with the loosening of adjacent teeth.
Odontogenic Fibroma
Histologic Features

Some authors have described two separate types of odontogenic fibromas.

The simple odontogenic fibroma is composed of stellate fibroblasts arranged in a whorled pattern with fine collagen fibrils and a lot of
ground substance.

Foci of odontogenic epithelium may or may not be present.

Occasionally, foci of dystrophic calcification may be present.

Odontogenic epithelium in the form of long strands or isolated nests is present throughout the lesion.

Calcifications composed of cementoid and/or dentinoid may be present.


Odontogenic Fibroma
Treatment and Prognosis

The odontogenic fibroma is usually treated by enucleation and curettage.

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


Odontogenic Myxoma
Clinical and Radiographic Features:

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

Gender Predilection: Reported to be about equal.

Location: May occur in any area of the jaws but more common in the mandible.

Radiographic Appearance:

Radiolucent lesion often with a multilocular appearance. The borders may be indistinct.
Odontogenic Myxoma
Odontogenic Myxoma
Histologic Features

The tumor is composed of loosely arranged

Stellate

Spindle-shaped

Round cells in - loose myxoid stroma with few collagen bundles.

Epithelial cells are not required for diagnosis.

May be confused with a chrondromyxoid fibroma or with myxoid change in an enlarged dental follicle or papilla.
Cementoblastoma
Clinical Features

Patient Age: This lesion is most commonly occurs in the 2nd and 3rd decades.

Gender Predilection: Approximately equal.

Location: The cementoblastoma is associated with the roots of posterior teeth and is more common in the mandible than the maxilla.

Radiologic Features:

Radiographically, the lesion appears as an opaque lesion attached to and replacing the root of the involved tooth.

Opaque spicules radiate from the central mass.


Cementoblastoma
Cementoblastoma

Histologic Features

The lesion is composed of sheets or thick trabeculae of mineralized material with irregularly placed lacunae and prominent basophilic reversal
lines. Multinucleated giant cells are often present.

This lesion closely resembles the osteoblastoma.


Cementoblastoma
Additional Features, Treatment and Prognosis

The cementoblastoma is a slow-growing lesion that may cause local expansion of the jaw.

Pain less and asymptomatic.

Treatment

Surgical extraction of the tooth together with the attached mass.

Prognosis is excellent as the lesion does not recur.


Surgical Options in management of Odontogenic
Tumours
Mandible: -
Benign Mandible Tumours Access incisions:
Type or surgery depends on number of factors
1. Intraoral transmucosal access
1. Type of pathology—Benign/Benign aggressive
2. Extent of involvement—e.g. Sufficient lower border of 2. Combined Intraoral and extraoral submandibular
mandible present or not access
3. 3.Site of tumour—Anterior mandible/Posterior mandible
3. Combined Intraoral and Visor incision
Based on this options starting from the most conservative to most
radical are: Repair:
1. Marsupialisation
1. Primary mucosal closure
2. Enucleation or curettage
3. Peripheral ostectomy or En bloc resection 2. Packing cavity and healing by secondary intention
4. Segmental resection
3. Reconstruction plate with primary mucosal closure

4. Reconstruction plate with free bone graft

5. Vascularised Bone free flap


Surgical Options in management of Odontogenic
Tumours
Maxilla: -

Type or surgery depends on number of factors

1. Type of pathology—Benign/Benign aggressive

2. Extent of involvement—e.g. Involvement of Access incisions:


sinus/pterygoids/infratemporal fossa
1. Transoral accesss
3. Site of tumour—Anterior maxilla/Posterior maxilla 2. Weber Ferguson incision—Good for anterior
tumours
Based on this the following excision options are possible: 3. Mandibulotomy access approach—This provides
1. Marsupialization into Oral cavity/Maxillary Sinus
excellent access for posterior tumours especially when access to the
pterygoids/intratemporal region is required
2. Enucleation/Curretage

3. Enbloc resection—Low level/High level


Repair:
maxillectomy 1. Primary closure
2. Packing cavity to allow healing by secondary intention with a Healing plate
3. Obturator
4. Local flap e.g. Temporalis
5. Free flap reconstruction
Surgical Options in management of Odontogenic
Tumours
Need for reconstruction:
1. For restoration of movements and equilibrium of mandible
2. For maintenance of normal occlusal plane, floor of the mouth and
tongue’s anatomical position Treatment Goal:
3. For restoration of near normal feeding Complete removal of tumour
4. For acceptable aesthetics and function. Aesthetic reconstruction with minimal disfigurement
5. For more favourable social acceptance Good prognosis
6. To establish the arch form, width and alveolar height. Long-term follow-up
7. To establish the bone continuity and maintain facial contours
Recent Advances in Molecular Diagnosis
Epithelial Tumors

Ameloblastoma

High frequency of BRAF V600E and SMO L412F mutations

This is followed by KRAS (mostly p.G12R), NRAS, HRAS, FGFR2, and mutations reported in a few BRAF wild- type cases.

Somatic mutations in KRAS, PIK3CA, PTEN, FGFR, CDKN2A, and CTNNB1

Adenomatoid Odontogenic Tumor

frequent KRAS codon 12 (either p.G12V or p.G12R, and in a single case p.G12D)
Recent Advances
Extra Oral Unit

Intraoral Unit

Extra Oral - Negative Pressure of 45 mmhg

Intra Oral - Irrigation and Decompression Tube

The distal end of both tube are outside the cavity

1 of the tubes have a needle port for lavage - usually saline or


chlorhexidine

2nd tube is connected to external vacuum

Secured to neighbouring teeth with orthodontic wires


Recent Advances
Active decompression with distraction sugosteogenesis (ADDS) has been successfully used for the treatment of conventional ameloblastoma.
Till now, ADDS use was limited to the conservative management of odontogenic cystic conditions. Wiscovitch et al. showed that ADDS can
prove to be a viable treatment because it demonstrates a reduction in the size of the initial lesion by new osseous formation within two weeks of
placement of the device.

After successful surgery for ameloblastoma, prevention of recurrence has been a long-standing issue. Recently, a machine learning algorithm has
been used to predict recurrence with reliable accuracy. The four most important variables influencing ameloblastoma recurrence were the time
elapsed from treatment, initial surgical treatment, tumor size, and radiographic presentation. These were used in the algorithm to reliably predict
recurrence.

Similarly, Yang et al. have developed a favourable nomogram that accurately predicted the recurrence-free survival of patients with
ameloblastoma based on individual characteristics, which could optimise tailored therapy and follow-up.
Recent Advances
Discovery and elucidation of the activated molecular pathways discussed helps in novel molecular targeted therapies in the management of
ameloblastoma with genetic mutations. The key benefit of molecular targeted therapies is that it can significantly reduce the surgical morbidity

The results showed that the model was able to predict recurrence of ameloblastoma with reliable accuracy. This study provides insights into the
detection of high-risk patient groups to monitor recurrence.

Yang et al in 2021 stated that the recurrence of ameloblastoma was significantly associated with Cortical Bone Perforation, WHO Classification,
and surgical treatment pattern
Recent Advances

Shi HA et al., Ameloblastoma:


A Succinct Review of the
classification, genetic
understanding and novel
molecular targeted therapies,
The Surgeon,
https://doi.org/10.1016/j.surge.
2020.06.009
Recent Advances
Non surgical treatment included

helical tomotherapy,

image guided radiation therapy,

intensity-modulated radiation therapy, and

proton beam therapy. Some of these treatment modalities have been combined with surgery and/or chemotherapy. The therapeutic use of adjuvant radiotherapy with or without
chemotherapy for positive margins of recurrent and unresectable ameloblastomas has resulted in mixed outcomes

The drugs which have potential to be used in molecular targeted therapies of ameloblastoma are those which inhibit the functions of mutated BRAF and MEK.

These are vemurafenib and dabrafenib, which inhibit mutated BRAF gene;

trametinib, which inhibits the mutated MEK gene; and

ponatinib and regorafenib which inhibit the mutated FGFR2 genes.


Textbook of Oral and Maxillofacial surgery for clinician by AOMSI
References
Textbook of oral and maxillofacial surgery by Neelima Anil Malik

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Ghai S. Ameloblastoma: an updated narrative review of an enigmatic tumor. Cureus. 2022 Aug 6;14(8).

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González-González R, López-Verdín S, Lavalle-Carrasco J, Molina-Frechero N, Isiordia-Espinoza M, Carreón-Burciaga RG, Bologna-Molina R. Current concepts in ameloblastoma-targeted therapies in B-raf proto-oncogene serine/threonine kinase V600E mutation: Systematic review. World journal of
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Yang YC, Wang JJ, Huang Y, Cai WX, Tao Q. Development and validation of a prognostic nomogram for postoperative recurrence-free survival of ameloblastoma. Cancer Management and Research. 2021 Jun 1:4403-16.

Ghafouri-Fard S, Atarbashi-Moghadam S, Taheri M. Genetic factors in the pathogenesis of ameloblastoma, dentigerous cyst and odontogenic keratocyst. Gene. 2021 Mar 1;771:145369.

Richardson MS, Muller S. Malignant odontogenic tumors: an update on selected tumors. Head and neck pathology. 2014 Dec;8:411-20.

Buettner R, Gültekin SE. Molecular diagnostics in odontogenic tumors. Die Pathologie. 2022 Dec;43(Suppl 1):81-5.

Farshbaf A, Zare R, Mohajertehran F, Mohtasham N. New diagnostic molecular markers and biomarkers in odontogenic tumors. Molecular biology reports. 2021 Apr;48:3617-28.

Rioux-Forker D, Deziel AC, Williams LS, Muzaffar AR. Odontogenic cysts and tumors. Annals of plastic surgery. 2019 Apr 1;82(4):469-77.

Kitisubkanchana J, Reduwan NH, Poomsawat S, Pornprasertsuk-Damrongsri S, Wongchuensoontorn C. Odontogenic keratocyst and ameloblastoma: radiographic evaluation. Oral radiology. 2021 Jan;37:55-65.

Ren C, Amm HM, DeVilliers P, Wu Y, Deatherage JR, Liu Z, MacDougall M. Targeting the sonic hedgehog pathway in keratocystic odontogenic tumor. Journal of Biological Chemistry. 2012 Aug 3;287(32):27117-25.

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